8896
Context
2-Hydroxyglutric Aciduria (D and L Form)
L-2-Hydroxyglutaric Aciduria (D-2-Hydroxyglutaric Aciduria ) is an autosomal recessive disease caused by a mutation in the L2HGDH gene which codes for L-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of L-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; and L-lysine in plasma and spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results. D-2-Hydroxyglutaric Aciduria is an autosomal recessive disease caused by a mutation in the D2HGDH gene which does for D-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of D-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; oxoglutaric acid in urine; and gabba-aminobutyric acid in spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results.
Disease
PW121689
Center
PathwayVisualizationContext121965
3300
2600
#000099
PathwayVisualization64311
64427
Glutamate Metabolism
Glutamate is one of the non-essential amino acids that is produced by the body. Glutamate is precursor for many nucleic acids and proteins in addition to its role in the central nervous system. It is an excitatory neurotransmitter and has a role in neuronal plasticity, affecting memory and learning. Glutamate plays a role in numerous metabolic pathways. Dysfunctional glutamate metabolism may cause disorders such as: gyrate atrophy, hyperammonemia, γ-hydoxybutyric aciduria, hemolytic anemia, and 5-oxoprolinuria.
Metabolic
12
107902
15
SubPathway
106941
1003
Compound
112
107903
73
SubPathway
106942
95
Compound
111
107904
6
SubPathway
106943
95
Compound
111
107905
78
SubPathway
106944
95
Compound
111
107906
100
SubPathway
106945
862
Compound
111
107907
91
SubPathway
106946
882
Compound
111
107908
15
SubPathway
106947
71
Compound
111
229612
Lehninger, A.L. Lehninger principles of biochemistry (4th ed.) (2005). New York: W.H Freeman.
64427
Pathway
229613
Salway, J.G. Metabolism at a glance (3rd ed.) (2004). Alden, Mass.: Blackwell Pub.
64427
Pathway
1
Cell
CL:0000000
2
Platelet
CL:0000233
5
Hepatocyte
CL:0000182
3
Neuron
CL:0000540
4
Cardiomyocyte
CL:0000746
8
Beta cell
CL:0000639
7
Epithelial Cell
CL:0000066
6
Myocyte
CL:0000187
1
Homo sapiens
9606
Eukaryote
Human
2
Bacteria
2
Prokaryote
Bacteria
3
Escherichia coli
562
Prokaryote
12
Mus musculus
10090
Eukaryote
Mouse
17
Rattus norvegicus
10116
Eukaryote
Rat
19
Schizosaccharomyces pombe
4896
Eukaryote
24
Solanum lycopersicum
4081
Eukaryote
Tomato
4
Arabidopsis thaliana
3702
Eukaryote
Thale cress
18
Saccharomyces cerevisiae
4932
Eukaryote
Yeast
21
Xenopus laevis
8355
Eukaryote
African clawed frog
6
Caenorhabditis elegans
6239
Eukaryote
Roundworm
25
Escherichia coli (strain K12)
83333
Prokaryote
49
Bathymodiolus platifrons
220390
Eukaryote
Deep sea mussel
23
Pseudomonas aeruginosa
287
Prokaryote
60
Nitzschia sp.
0001
Eukaryote
Nitzschia4
5
Bos taurus
9913
Eukaryote
Cattle
10
Drosophila melanogaster
7227
Eukaryote
Fruit fly
29
Saccharomyces cerevisiae (strain ATCC 204508 / S288c)
559292
Eukaryote
Baker's yeast
51
Picea sitchensis
3332
Eukaryote
Sitka spruce
1
Cytosol
GO:0005829
3
Mitochondrial Matrix
GO:0005759
5
Cytoplasm
GO:0005737
14
Mitochondrial Outer Membrane
GO:0005741
2
Mitochondrion
GO:0005739
15
Nucleus
GO:0005634
4
Peroxisome
GO:0005777
13
Endoplasmic Reticulum
GO:0005783
7
Endoplasmic Reticulum Membrane
GO:0005789
10
Cell Membrane
GO:0005886
27
Peroxisome Membrane
GO:0005778
31
Periplasmic Space
GO:0005620
11
Extracellular Space
GO:0005615
35
Chloroplast
GO:0009507
12
Mitochondrial Inner Membrane
GO:0005743
32
Inner Membrane
GO:0070258
6
Lysosome
GO:0005764
24
Mitochondrial Intermembrane Space
GO:0005758
19
sarcoplasmic reticulum
GO:0016529
34
Plant-Type Vacuole
GO:0000325
25
Golgi apparatus
GO:0005794
26
Golgi apparatus membrane
GO:0000139
16
Lysosomal Lumen
GO:0043202
18
Melanosome Membrane
GO:0033162
20
Endoplasmic Reticulum Lumen
GO:0005788
21
Synapse
GO:0045202
36
Membrane
GO:0016020
53
Endoplasmic Reticulum Body
GO:0010168
40
Periplasm
GO:0042597
8
Smooth Endoplasmic Reticulum
GO:0005790
39
Mitochondrial membrane
GO:0031966
37
Basolateral cell membrane
GO:0016323
2
Endothelium
BTO:0000393
1
Liver
BTO:0000759
72
9
7
Nervous System
BTO:0001484
18
Pancreas
BTO:0000988
25
Intestine
BTO:0000648
8
Blood Vessel
BTO:0001102
74
11
9
Muscle
BTO:0000887
141
18
24
Brain
BTO:0000142
89
16
5
cardiocyte
BTO:0001539
4
Adrenal Medulla
BTO:0000049
71
8
28
Stomach
BTO:0001307
155
26
11
Heart
BTO:0000562
73
10
3
Sympathetic Nervous System
BTO:0001832
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Adenosine triphosphate
HMDB0000538
Adenosine triphosphate (ATP) is a nucleotide consisting of a purine base (adenine) attached to the first carbon atom of ribose (a pentose sugar). Three phosphate groups are esterified at the fifth carbon atom of the ribose. ATP is incorporated into nucleic acids by polymerases in the processes of DNA replication and transcription. ATP contributes to cellular energy charge and participates in overall energy balance, maintaining cellular homeostasis. ATP can act as an extracellular signaling molecule via interactions with specific purinergic receptors to mediate a wide variety of processes as diverse as neurotransmission, inflammation, apoptosis, and bone remodelling. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin, and ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity. During exercise, intracellular homeostasis depends on the matching of adenosine triphosphate (ATP) supply and ATP demand. Metabolites play a useful role in communicating the extent of ATP demand to the metabolic supply pathways. Effects as different as proliferation or differentiation, chemotaxis, release of cytokines or lysosomal constituents, and generation of reactive oxygen or nitrogen species are elicited upon stimulation of blood cells with extracellular ATP. The increased concentration of adenosine triphosphate (ATP) in erythrocytes from patients with chronic renal failure (CRF) has been observed in many studies but the mechanism leading to these abnormalities still is controversial. (PMID: 15490415, 15129319, 14707763, 14696970, 11157473).
56-65-5
C00002
5957
15422
ATP
5742
DB00171
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
C10H16N5O13P3
InChI=1S/C10H16N5O13P3/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(26-10)1-25-30(21,22)28-31(23,24)27-29(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H,23,24)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1
ZKHQWZAMYRWXGA-KQYNXXCUSA-N
({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)phosphonic acid
507.181
506.995745159
-2.05
7
adenosine triphosphate
0
-3
FDB021813
5'-(tetrahydrogen triphosphate) adenosine;5'-atp;Atp;Adenosine 5'-triphosphate;Adenosine 5'-triphosphorate;Adenosine 5'-triphosphoric acid;Adenosine triphosphate;Adenylpyrophosphorate;Adenylpyrophosphoric acid;Adephos;Adetol;Adynol;Atipi;Atriphos;Cardenosine;Fosfobion;Glucobasin;Myotriphos;Phosphobion;Striadyne;Triadenyl;Triphosphaden;Triphosphoric acid adenosine ester;Adenosine-5'-triphosphate;H4atp;Adenosine triphosphoric acid;Adenosine-5'-triphosphoric acid
PW_C000414
ATP
9
2
21
4
60
8
266
16
414
22
478
13
733
32
799
5
934
39
976
3
2105
18
2112
10
2146
49
2156
14
2160
58
2405
59
2434
27
2726
46
2812
29
3029
66
3163
72
3616
61
3617
51
4399
23
4474
31
4768
91
4864
54
5032
89
5035
26
5155
7
5205
97
5215
100
5250
104
5291
101
5313
111
5346
112
5390
103
5406
117
5430
118
5443
120
5542
129
5556
132
5569
133
5603
135
5621
108
5846
143
5854
146
5876
107
5897
147
5924
151
6048
155
6109
161
6230
166
6493
178
6839
188
6870
160
6976
199
7157
205
7184
206
7209
210
7225
213
7229
211
7298
198
7302
216
7390
217
7408
218
7432
163
7481
222
7499
190
8186
225
11847
277
11903
170
12010
281
12039
164
12178
285
12578
226
12691
290
13264
223
15327
308
42326
315
42621
322
42694
318
77028
253
77218
134
77233
329
77468
333
77632
336
78037
332
78041
350
78168
128
78214
351
78240
353
78411
335
78494
115
78850
130
78865
331
78919
334
80028
368
80046
184
80674
119
85629
1
94826
124
113234
94
113282
388
116280
109
119914
122
119992
406
120154
407
120245
382
120362
412
121246
429
121392
123
121397
433
121471
408
121974
410
122065
125
122079
383
122083
405
122402
422
122444
435
122919
399
123009
446
123816
464
123951
447
123956
468
124029
374
124527
444
124616
136
124630
398
124634
376
124943
472
124972
375
125011
470
125304
297
125371
479
125392
299
125515
481
125595
484
126123
485
126220
300
126234
495
126240
478
126547
491
126596
499
126913
501
127123
389
127731
516
127781
395
127796
390
127801
209
128119
508
128167
517
95
L-Glutamic acid
HMDB0000148
Glutamic acid (Glu), also referred to as glutamate (the anion), is one of the 20 proteinogenic amino acids. It is not among the essential amino acids. Glutamate is a key molecule in cellular metabolism. In humans, dietary proteins are broken down by digestion into amino acids, which serves as metabolic fuel or other functional roles in the body. Glutamate is the most abundant fast excitatory neurotransmitter in the mammalian nervous system. At chemical synapses, glutamate is stored in vesicles. Nerve impulses trigger release of glutamate from the pre-synaptic cell. In the opposing post-synaptic cell, glutamate receptors, such as the NMDA receptor, bind glutamate and are activated. Because of its role in synaptic plasticity, it is believed that glutamic acid is involved in cognitive functions like learning and memory in the brain. Glutamate transporters are found in neuronal and glial membranes. They rapidly remove glutamate from the extracellular space. In brain injury or disease, they can work in reverse and excess glutamate can accumulate outside cells. This process causes calcium ions to enter cells via NMDA receptor channels, leading to neuronal damage and eventual cell death, and is called excitotoxicity. The mechanisms of cell death include: * Damage to mitochondria from excessively high intracellular Ca2+. * Glu/Ca2+-mediated promotion of transcription factors for pro-apoptotic genes, or downregulation of transcription factors for anti-apoptotic genes. Excitotoxicity due to glutamate occurs as part of the ischemic cascade and is associated with stroke and diseases like amyotrophic lateral sclerosis, lathyrism, and Alzheimer's disease. glutamic acid has been implicated in epileptic seizures. Microinjection of glutamic acid into neurons produces spontaneous depolarization around one second apart, and this firing pattern is similar to what is known as paroxysmal depolarizing shift in epileptic attacks. This change in the resting membrane potential at seizure foci could cause spontaneous opening of voltage activated calcium channels, leading to glutamic acid release and further depolarization. (http://en.wikipedia.org/wiki/Glutamic_acid).
56-86-0
C00025
33032
16015
GLT
30572
DB00142
N[C@@H](CCC(O)=O)C(O)=O
C5H9NO4
InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1
WHUUTDBJXJRKMK-VKHMYHEASA-N
(2S)-2-aminopentanedioic acid
147.1293
147.053157781
-0.26
3
L-glutamic acid
0
-1
FDB012535
(2s)-2-aminopentanedioate;(2s)-2-aminopentanedioic acid;(s)-(+)-glutamate;(s)-(+)-glutamic acid;(s)-2-aminopentanedioate;(s)-2-aminopentanedioic acid;(s)-glutamate;(s)-glutamic acid;1-amino-propane-1,3-dicarboxylate;1-amino-propane-1,3-dicarboxylic acid;1-aminopropane-1,3-dicarboxylate;1-aminopropane-1,3-dicarboxylic acid;2-aminoglutarate;2-aminoglutaric acid;2-aminopentanedioate;2-aminopentanedioic acid;Aciglut;Aminoglutarate;Aminoglutaric acid;E;Glt;Glu;Glusate;Glut;Glutacid;Glutamicol;Glutamidex;Glutaminate;Glutaminic acid;Glutaminol;Glutaton;L-(+)-glutamate;L-(+)-glutamic acid;L-glu;L-glutamate;L-glutaminate;L-glutaminic acid;L-a-aminoglutarate;L-a-aminoglutaric acid;L-alpha-aminoglutarate;L-alpha-aminoglutaric acid;A-aminoglutarate;A-aminoglutaric acid;A-glutamate;A-glutamic acid;Alpha-aminoglutarate;Alpha-aminoglutaric acid;Alpha-glutamate;Alpha-glutamic acid;Acide glutamique;Acido glutamico;Acidum glutamicum;Glutamate;Glutamic acid;L-glutaminsaeure
PW_C000095
Glu
16
2
44
3
65
8
119
1
138
4
164
14
969
9
1105
42
1448
50
1456
26
1462
54
5323
111
5344
113
5415
117
5439
118
5565
132
5631
107
5632
108
5859
105
6006
147
6071
157
6191
94
6531
85
6838
187
6844
188
7092
72
7093
71
7165
205
7182
207
7514
224
7518
151
8208
225
8373
220
11792
198
11855
161
12004
222
12621
31
12683
289
12697
290
42348
315
42349
318
42845
320
77020
253
77332
133
77525
112
77971
346
77977
327
77981
347
78291
345
80649
135
120023
124
120040
122
120086
407
120347
406
120692
126
120816
418
121147
423
121153
424
121157
425
122833
119
122997
120
123299
443
123401
454
123719
458
123725
459
123729
460
125401
299
125418
297
125457
481
125667
479
125769
301
125802
489
126941
388
126995
206
127162
501
127257
506
35
Ammonia
HMDB0000051
Ammonia is a colourless alkaline gas and is one of the most abundant nitrogen-containing compounds in the atmosphere. It is an irritant with a characteristic pungent odor that is widely used in industry. Inasmuch as ammonia is highly soluble in water and, upon inhalation, is deposited in the upper airways, occupational exposures to ammonia have commonly been associated with sinusitis, upper airway irritation, and eye irritation. Acute exposures to high levels of ammonia have also been associated with diseases of the lower airways and interstitial lung. Small amounts of ammonia are naturally formed in nearly all tissues and organs of the vertebrate organism. Ammonia is both a neurotoxin and a metabotoxin. In fact, it is the most common endogenous neurotoxin. A neurotoxin is a compound that causes damage to neural tissue and neural cells. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Ammonia is recognized to be central in the pathogenesis of a brain condition known as hepatic encephalopathy, which arises from various liver diseases and leads to a build up ammonia in the blood (hyperammonemia). More than 40% of people with cirrhosis develop hepatic encephalopathy. Part of the neurotoxicity of ammonia arises from the fact that it easily crosses the blood-brain barrier and is absorbed and metabolized by the astrocytes, a population of cells in the brain that constitutes 30% of the cerebral cortex. Astrocytes use ammonia when synthesizing glutamine from glutamate. The increased levels of glutamine lead to an increase in osmotic pressure in the astrocytes, which become swollen. There is increased activity of the inhibitory gamma-aminobutyric acid (GABA) system, and the energy supply to other brain cells is decreased. This can be thought of as an example of brain edema. The source of the ammonia leading to hepatic encephalopathy is not entirely clear. The gut produces ammonia, which is metabolized in the liver, and almost all organ systems are involved in ammonia metabolism. Colonic bacteria produce ammonia by splitting urea and other amino acids, however this does not fully explain hyperammonemia and hepatic encephalopathy. The alternative explanation is that hyperammonemia is the result of the intestinal breakdown of amino acids, especially glutamine. The intestines have significant glutaminase activity, predominantly located in the enterocytes. On the other hand, intestinal tissues only have a little glutamine synthetase activity, making it a major glutamine-consuming organ. In addition to the intestine, the kidney is an important source of blood ammonia in patients with liver disease. Ammonia is also taken up by the muscle and brain in hepatic coma, and there is confirmation that ammonia is metabolized in muscle. Excessive formation of ammonia in the brains of Alzheimer's disease patients has also been demonstrated, and it has been shown that some Alzheimer's disease patients exhibit elevated blood ammonia concentrations. Ammonia is the most important natural modulator of lysosomal protein processing. Indeed, there is strong evidence for the involvement of aberrant lysosomal processing of beta-amyloid precursor protein (beta-APP) in the formation of amyloid deposits. Inflammatory processes and activation of microglia are widely believed to be implicated in the pathology of Alzheimer's disease. Ammonia is able to affect the characteristic functions of microglia, such as endocytosis, and cytokine production. Based on these facts, an ammonia-based hypothesis for Alzheimer's disease has been suggested (PMID: 17006913, 16167195, 15377862, 15369278). Chronically high levels of ammonia in the blood are associated with nearly twenty different inborn errors of metabolism including: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, 3-methyl-crotonylglycinuria, argininemia, argininosuccinic aciduria, beta-ketothiolase deficiency, biotinidase deficiency, carbamoyl phosphate synthetase deficiency, carnitine-acylcarnitine translocase deficiency, citrullinemia type I, hyperinsulinism-hyperammonemia syndrome, hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, isovaleric aciduria, lysinuric protein intolerance, malonic aciduria, methylmalonic aciduria, methylmalonic aciduria due to cobalamin-related disorders, propionic acidemia, pyruvate carboxylase deficiency, and short chain acyl CoA dehydrogenase deficiency (SCAD deficiency). Many of these inborn errors of metabolism are associated with urea cycle disorders or impairment of amino acid metabolism. High levels of ammonia in the blood (hyperammonemia) lead to the activation of NMDA receptors in the brain. This results in the depletion of brain ATP, which in turn leads to the release of glutamate. Ammonia also leads to the impairment of mitochondrial function and calcium homeostasis, thereby decreasing ATP synthesis. Excess ammonia also increases the formation of nitric oxide (NO), which in turn reduces the activity of glutamine synthetase, and thereby decreases the elimination of ammonia in the brain (PMID: 12020609). As a neurotoxin, ammonia predominantly affects astrocytes. Disturbed mitochondrial function and oxidative stress, factors implicated in the induction of the mitochondrial permeability transition, appear to be involved in the mechanism of ammonia neurotoxicity. Ammonia can also affect the glutamatergic and GABAergic neuronal systems, the two prevailing neuronal systems of the cortical structures. All of these effects can lead to irreversible brain damage, coma, and/or death. Infants with urea cycle disorders and hyperammonemia initially exhibit vomiting and increasing lethargy. If untreated, seizures, hypotonia (poor muscle tone, floppiness), respiratory distress (respiratory alkalosis), and coma can occur. Adults with urea cycle disorders and hyperammonemia will exhibit episodes of disorientation, confusion, slurred speech, unusual and extreme combativeness or agitation, stroke-like symptoms, lethargy, and delirium. Ammonia also has toxic effects when an individual is exposed to ammonia solutions. Acute exposure to high levels of ammonia in air may be irritating to skin, eyes, throat, and lungs and cause coughing and burns. Lung damage and death may occur after exposure to very high concentrations of ammonia. Swallowing concentrated solutions of ammonia can cause burns in the mouth, throat, and stomach. Splashing ammonia into eyes can cause burns and even blindness.
7664-41-7
C00014
222
16134
AMMONIA
217
N
H3N
InChI=1S/H3N/h1H3
QGZKDVFQNNGYKY-UHFFFAOYSA-N
ammonia
17.0305
17.026549101
1
ammonia
0
1
FDB003908
Ammonia anhydrous;Ammonia inhalant;Ammonia solution strong [usan];Ammonia water;Ammoniak;Liquid ammonia;Am-fol;Ammonia;Ammonia (conc 20% or greater);Ammonia gas;Ammonia solution;Ammonia solution strong (nf);Ammonia water (jp15);Ammoniac [french];Ammoniaca [italian];Ammoniacum gummi;Ammoniak [german];Ammoniak kconzentrierter;Ammoniakgas;Ammonium ion;Amoniak [polish];Anhydrous ammonia;Aromatic ammonia vaporole;Azane;Nh(3);Nh3;Nitro-sil;Primaeres amin;Sekundaeres amin;Spirit of hartshorn;Tertiaeres amin;[nh3];Ammoniac;Amoniaco;R-717;Ammonia solution strong
PW_C000035
NH3
97
9
112
5
133
8
142
4
438
2
479
13
550
14
1468
54
2533
22
2572
3
5338
111
6016
147
7022
160
7177
205
11786
198
11848
277
11885
215
12708
291
12718
292
76966
225
77046
294
77329
133
77343
132
77469
333
77499
113
77539
334
77597
115
77985
347
77993
112
78072
329
79244
293
80650
135
80657
119
116203
109
119921
122
120049
408
120053
126
120136
407
120343
406
120363
412
120462
405
121046
124
121161
425
122119
382
122800
374
122805
443
122993
120
123010
446
123096
376
123610
118
123733
460
124671
399
125311
297
125427
482
125431
301
125502
481
125663
479
125708
478
126102
299
126274
484
126966
502
126970
207
127039
206
127158
501
127200
209
127600
388
127837
389
1034
Adenosine diphosphate
HMDB0001341
Adenosine diphosphate, abbreviated ADP, is a nucleotide. It is an ester of pyrophosphoric acid with the nucleotide adenine. ADP consists of the pyrophosphate group, the pentose sugar ribose, and the nucleobase adenine. ADP is the product of ATP dephosphorylation by ATPases. ADP is converted back to ATP by ATP synthases.
58-64-0
C00008
6022
16761
ADP
5800
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
C10H15N5O10P2
InChI=1S/C10H15N5O10P2/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(24-10)1-23-27(21,22)25-26(18,19)20/h2-4,6-7,10,16-17H,1H2,(H,21,22)(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1
XTWYTFMLZFPYCI-KQYNXXCUSA-N
[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]phosphonic acid
427.2011
427.029414749
-2.12
6
adenosine-diphosphate
0
-2
FDB021817
Adp;Adenosindiphosphorsaeure;Adenosine 5'-pyrophosphate;Adenosine diphosphate;Adenosine pyrophosphate;Adenosine-5'-diphosphate;Adenosine-5-diphosphate;Adenosine-diphosphate;5'-adenylphosphoric acid;Adenosine 5'-diphosphate;H3adp;5'-adenylphosphate;Adenosine 5'-diphosphoric acid;Adenosine-5'-diphosphoric acid
PW_C001034
ADP
23
4
134
8
415
22
482
13
801
5
963
15
978
3
1061
14
1518
2
1901
49
2104
18
2113
10
2161
58
2408
59
2435
27
2728
47
2736
46
2855
29
3165
72
3635
61
4400
23
4476
31
4770
91
5036
26
5157
7
5208
97
5217
100
5315
111
5349
112
5392
103
5446
120
5544
129
5572
133
5624
108
5741
117
5764
101
5849
143
5856
146
5878
107
5899
147
5926
151
6050
155
6111
161
6231
166
6495
178
6700
94
6841
188
6872
160
7159
205
7187
206
7208
210
7226
213
7231
211
7300
198
7303
216
7391
217
7410
218
7433
163
7483
222
8187
225
11851
277
11905
170
12013
281
12180
285
13262
223
15329
308
42328
315
42398
313
42622
322
42696
318
77029
253
77087
132
77216
134
77306
329
77472
333
77663
336
78039
332
78043
350
78170
128
78215
351
78244
353
78414
335
78495
115
78705
331
78849
130
78920
334
80030
368
80622
118
80651
135
80676
119
94827
124
113283
388
116204
109
119944
122
119994
406
120156
407
120318
382
120366
412
121248
429
121394
123
121399
433
121472
408
121899
383
121976
410
122064
125
122085
405
122405
422
122445
435
122973
399
123013
446
123818
464
123953
447
123958
468
124030
374
124452
398
124529
444
124615
136
124636
376
124947
472
124975
375
125012
470
125334
297
125373
479
125492
299
125517
481
125645
484
126125
485
126219
300
126235
495
126242
478
126550
491
126597
499
126915
501
127733
516
127780
395
127797
390
127803
209
128122
508
128168
517
128313
389
1104
Phosphate
HMDB0001429
Phosphate is a salt of phosphoric acid. In organic chemistry, a phosphate, or organophosphate, is an ester of phosphoric acid. Organic phosphates are important in biochemistry, biogeochemistry and ecology. Phosphate (Pi) is an essential component of life. In biological systems, phosphorus is found as a free phosphate ion in solution and is called inorganic phosphate, to distinguish it from phosphates bound in various phosphate esters. Inorganic phosphate is generally denoted Pi and at physiological (neutral) pH primarily consists of a mixture of HPO<sup>2-</sup><sub>4</sub> and H<sub>2</sub>PO<sup>-</sup><sub>4</sub> ions. phosphates are most commonly found in the form of adenosine phosphates, (AMP, ADP and ATP) and in DNA and RNA and can be released by the hydrolysis of ATP or ADP. Similar reactions exist for the other nucleoside diphosphates and triphosphates. Phosphoanhydride bonds in ADP and ATP, or other nucleoside diphosphates and triphosphates, contain high amounts of energy which give them their vital role in all living organisms. Phosphate must be actively transported into cells against its electrochemical gradient. In vertebrates, two unrelated families of Na+-dependent Pi transporters carry out this task. Remarkably, the two families transport different Pi species: whereas type II Na+/Pi cotransporters (SCL34) prefer divalent HPO4(2), type III Na+/Pi cotransporters (SLC20) transport monovalent H2PO4. The SCL34 family comprises both electrogenic and electroneutral members that are expressed in various epithelia and other polarized cells. Through regulated activity in apical membranes of the gut and kidney, they maintain body Pi homeostasis, and in salivary and mammary glands, liver, and testes they play a role in modulating the Pi content of luminal fluids. Phosphate levels in the blood play an important role in hormone signaling and in bone homeostasis. In classical endocrine regulation, low serum phosphate induces the renal production of the seco-steroid hormone 1,25-dihydroxyvitamin D3 (1,25(OH)2D3).This active metabolite of vitamin D acts to restore circulating mineral (i.e. phosphate and calcium) levels by increasing absorption in the intestine, reabsorption in the kidney, and mobilization of calcium and phosphate from bone. Thus, chronic renal failure is associated with hyperparathyroidism, which in turn contributes to osteomalacia (softening of the bones). Another complication of chronic renal failure is hyperphosphatemia (low levels of phosphate in the blood). Hyperphosphatemia (excess levels of phosphate in the blood) is a prevalent condition in kidney dialysis patients and is associated with increased risk of mortality. Hypophosphatemia (hungry bone syndrome) has been associated to postoperative electrolyte aberrations and after parathyroidectomy. (PMID: 17581921, 11169009, 11039261, 9159312, 17625581)Fibroblast growth factor 23 (FGF-23) has recently been recognized as a key mediator of phosphate homeostasis, its most notable effect being promotion of phosphate excretion. FGF-23 was discovered to be involved in diseases such as autosomal dominant hypophosphatemic rickets, X-linked hypophosphatemia, and tumor-induced osteomalacia in which phosphate wasting was coupled to inappropriately low levels of 1,25(OH)2D3. FGF-23 is regulated by dietary phosphate in humans. In particular it was found that phosphate restriction decreased FGF-23, and phosphate loading increased FGF-23.
14265-44-2
C00009
1061
18367
CPD-8587
1032
[O-]P([O-])([O-])=O
O4P
InChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)/p-3
NBIIXXVUZAFLBC-UHFFFAOYSA-K
phosphoric acid
94.9714
94.95342
3
phosphoric acid
0
-2
DBMET00532
FDB022617
Nfb orthophosphate;O-phosphoric acid;Ortho-phosphate;Orthophosphate (po43-);Orthophosphate(3-);Phosphate;Phosphate (po43-);Phosphate anion(3-);Phosphate ion (po43-);Phosphate ion(3-);Phosphate trianion;Phosphate(3-);Phosphoric acid ion(3-);Pi;[po4](3-);Orthophosphate;Phosphate ion;Po4(3-);Phosphoric acid;Orthophosphoric acid;Phosphoric acid ion
PW_C001104
Pi
24
4
84
8
814
5
818
18
831
2
980
3
1763
14
1767
49
2500
10
2729
47
2737
46
3129
29
3166
72
3636
61
3851
23
4249
22
4475
31
5031
27
5158
7
5207
97
5216
100
5317
111
5351
112
5381
103
5447
120
5543
129
5573
133
5605
135
5625
108
5693
6
5848
143
5855
146
5911
147
5941
151
6040
155
6100
161
6294
107
6487
178
6691
101
6714
117
6842
188
6889
160
7161
205
7189
206
7212
211
7306
198
7389
210
7402
212
7436
163
7475
222
8196
225
8258
227
10118
241
10134
257
11748
132
11761
115
11773
213
11904
170
11927
164
12014
281
12728
290
13263
223
34819
17
42255
304
42350
315
42435
318
43692
322
77018
253
77194
293
77217
134
77940
336
77966
130
78048
332
78057
329
78245
353
78669
331
80022
368
89279
308
93831
383
94796
384
110558
390
110640
391
113235
94
115845
398
116206
109
119982
406
120069
122
120699
407
121057
124
121216
125
121268
429
121352
121
121409
123
121423
382
121852
405
123304
119
123621
118
123786
136
123838
464
123968
447
123981
399
124405
376
124948
472
125362
479
125446
297
125774
481
125954
299
126221
478
126594
300
126604
298
126723
484
126904
501
127413
388
127783
209
128166
395
128177
513
128315
389
500
L-Glutamine
HMDB0000641
Glutamine (Gln) is one of the 20 amino acids encoded by the standard genetic code. Its side chain is an amide; it is formed by replacing a side-chain hydroxyl of glutamic acid with an amine functional group. glutamine is found in foods high in proteins, such as fish, red meat, beans, and dairy products. glutamine is a supplement that is used in weightlifting, bodybuilding, endurance and other sports, as well as by those who suffer from muscular cramps or pain particularly elderly people. The main use of glutamine within the diet of either group is as a means of replenishing the body's stores of amino acids that have been used during exercise or everyday activities. Studies which are looking into problems with excessive consumption of glutamine thus far have proved inconclusive. However, normal supplementation is healthy mainly because glutamine is supposed to be supplemented after prolonged periods of exercise (for example, a workout or exercise in which amino acids are required for use) and replenishes amino acid stores; this being the main reason glutamine is recommended during fasting or for people who suffer from physical trauma, immune deficiencies, or cancer. There is a significant body of evidence that links glutamine-enriched diets with intestinal effects; aiding maintenance of gut barrier function, intestinal cell proliferation and differentiation, as well as generally reducing septic morbidity and the symptoms of Irritable Bowel Syndrome. The reason for such "cleansing" properties is thought to stem from the fact that the intestinal extraction rate of glutamine is higher than that for other amino acids, and is therefore thought to be the most viable option when attempting to alleviate conditions relating to the gastrointestinal tract. These conditions were discovered after comparing plasma concentration within the gut between glutamine-enriched and non glutamine-enriched diets. However, even though glutamine is thought to have "cleansing" properties and effects, it is unknown to what extent glutamine has clinical benefits, due to the varied concentrations of glutamine in varieties of food. It is also known that glutamine has various effects in reducing healing time after operations. Hospital waiting times after abdominal surgery are reduced by providing parenteral nutrition regimens containing amounts of glutamine to patients. Clinical trials have revealed that patients on supplementation regimes containing glutamine have improved nitrogen balances, generation of cysteinyl-leukotrienes from polymorphonuclear neutrophil granulocytes and improved lymphocyte recovery and intestinal permeability (in postoperative patients) - in comparison to those who had no glutamine within their dietary regime; all without any side-effects. (http://en.wikipedia.org/wiki/glutamine).
56-85-9
C00064
5961
18050
GLN
5746
DB00130
N[C@@H](CCC(N)=O)C(O)=O
C5H10N2O3
InChI=1S/C5H10N2O3/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H2,7,8)(H,9,10)/t3-/m0/s1
ZDXPYRJPNDTMRX-VKHMYHEASA-N
(2S)-2-amino-4-carbamoylbutanoic acid
146.1445
146.069142196
-0.17
3
L-glutamine
0
0
FDB012164
(2s)-2,5-diamino-5-oxopentanoate;(2s)-2,5-diamino-5-oxopentanoic acid;(2s)-2-amino-4-carbamoylbutanoate;(2s)-2-amino-4-carbamoylbutanoic acid;(s)-2,5-diamino-5-oxopentanoate;(s)-2,5-diamino-5-oxopentanoic acid;2-aminoglutaramic acid;Cebrogen;Glavamin;Glumin;Glutamic acid 5-amide;Glutamic acid amide;Glutamine;L-(+)-glutamine;L-2-aminoglutaramic acid;L-2-aminoglutaramidic acid;L-glutamic acid 5-amide;L-glutamic acid gamma-amide;L-glutamid;L-glutamide;L-glutamin;L-glutamine;L-glutaminsaeure-5-amid;Levoglutamid;Levoglutamida;Levoglutamide;Levoglutamidum;Levoglutamina;Polyglutamine;Stimulina;Gamma-glutamine;Q;Glutamate 5-amide;Glutamate amide
PW_C000500
Gln
61
8
443
4
2701
2
3021
15
5658
107
5659
108
6068
157
6846
166
6847
3
8390
225
11793
198
12692
290
42352
318
42353
315
77016
253
77296
111
77333
133
78395
132
79164
114
79264
116
80652
135
113207
94
116207
109
120038
122
120348
406
121219
409
121961
124
122998
120
123789
137
124514
118
125416
297
125668
479
126359
299
126812
483
126956
205
127163
501
127718
388
128411
208
20
Biotin
HMDB0000030
Biotin is an enzyme co-factor present in minute amounts in every living cell. Biotin is also known as vitamin H or B7 or coenzyme R. It occurs mainly bound to proteins or polypeptides and is abundant in liver, kidney, pancreas, yeast, and milk. Biotin has been recognized as an essential nutrient. Our biotin requirement is fulfilled in part through diet, through endogenous reutilization of biotin and perhaps through capture of biotin generated in the intestinal flora. The utilization of biotin for covalent attachment to carboxylases and its reutilization through the release of carboxylase biotin after proteolytic degradation constitutes the 'biotin cycle'. Biotin deficiency is associated with neurological manifestations, skin rash, hair loss and metabolic disturbances that are thought to relate to the various carboxylase deficiencies (metabolic ketoacidosis with lactic acidosis). It has also been suggested that biotin deficiency is associated with protein malnutrition, and that marginal biotin deficiency in pregnant women may be teratogenic. Biotin acts as a carboxyl carrier in carboxylation reactions. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lysine residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. There are four biotin-dependent carboxylases in mammals: those of propionyl-CoA (PCC), 3-methylcrotonyl-CoA (MCC), pyruvate (PC) and acetyl-CoA carboxylases (isoforms ACC-1 and ACC-2). All but ACC-2 are mitochondrial enzymes. The biotin moiety is covalently bound to the epsilon amino group of a Lys residue in each of these carboxylases in a domain 60-80 amino acids long. The domain is structurally similar among carboxylases from bacteria to mammals. Evidence is emerging that biotin participates in processes other than classical carboxylation reactions. Specifically, novel roles for biotin in cell signaling, gene expression, and chromatin structure have been identified in recent years. Human cells accumulate biotin by using both the sodium-dependent multivitamin transporter and monocarboxylate transporter 1. These transporters and other biotin-binding proteins partition biotin to compartments involved in biotin signaling: cytoplasm, mitochondria, and nuclei. The activity of cell signals such as biotinyl-AMP, Sp1 and Sp3, nuclear factor (NF)-kappaB, and receptor tyrosine kinases depends on biotin supply. Consistent with a role for biotin and its catabolites in modulating these cell signals, greater than 2000 biotin-dependent genes have been identified in various human tissues. Many biotin-dependent gene products play roles in signal transduction and localize to the cell nucleus, consistent with a role for biotin in cell signaling. Posttranscriptional events related to ribosomal activity and protein folding may further contribute to effects of biotin on gene expression. Finally, research has shown that biotinidase and holocarboxylase synthetase mediate covalent binding of biotin to histones (DNA-binding proteins), affecting chromatin structure; at least seven biotinylation sites have been identified in human histones. Biotinylation of histones appears to play a role in cell proliferation, gene silencing, and the cellular response to DNA repair. Roles for biotin in cell signaling and chromatin structure are consistent with the notion that biotin has a unique significance in cell biology. (PMID: 15992684, 16011464).
58-85-5
C00120
171548
15956
BIOTIN
149962
DB00121
[H][C@]12CS[C@@H](CCCCC(O)=O)[C@@]1([H])NC(=O)N2
C10H16N2O3S
InChI=1S/C10H16N2O3S/c13-8(14)4-2-1-3-7-9-6(5-16-7)11-10(15)12-9/h6-7,9H,1-5H2,(H,13,14)(H2,11,12,15)/t6-,7-,9-/m0/s1
YBJHBAHKTGYVGT-ZKWXMUAHSA-N
5-[(3aS,4S,6aR)-2-oxo-hexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoic acid
244.311
244.088163078
-2.30
3
5-[(3aS,4S,6aR)-2-oxo-hexahydrothieno[3,4-d]imidazol-4-yl]pentanoic acid
0
-1
FDB014510
(+)-biotin;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valerate;(+)-cis-hexahydro-2-oxo-1h-thieno[3,4]imidazole-4-valeric acid;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valerate;(3as,4s,6ar)-hexahydro-2-oxo-1h-thieno[3,4-d]imidazole-4-valeric acid;-(+)-biotin;1swk;1swn;1swr;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoate;5-(2-oxohexahydro-1h-thieno[3,4-d]imidazol-4-yl)pentanoic acid;Biodermatin;Bioepiderm;Bios ii;Bios h;Biotin;Coenzyme r;D(+)-biotin;D-(+)-biotin;D-biotin;D-biotin factor s;Factor s;Factor s (vitamin);Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoate;Hexahydro-2-oxo-1h-thieno(3,4-d)imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3aa,4b,6aa)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoate;Hexahydro-2-oxo-[3as-(3alpha,4beta,6alpha)]-1h-thieno[3,4-d]imidazole-4-pentanoic acid;Lutavit h2;Meribin;Rovimix h 2;Vitamin b7;Vitamin h;Vitamin-h;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valerate;Cis-(+)-tetrahydro-2-oxothieno[3,4]imidazoline-4-valeric acid;Cis-hexahydro-2-oxo-1h-thieno(3,4)imidazole-4-valeric acid;Cis-tetrahydro-2-oxothieno(3,4-d)imidazoline-4-valeric acid;Delta-(+)-biotin;Delta-biotin;Delta-biotin factor s;Biotina;Biotine;Biotinum
PW_C000020
Biotin
26
4
135
8
579
15
1699
3
2270
2
5292
101
5298
105
5393
103
5449
120
5546
111
5551
114
5575
133
6051
155
6112
161
6496
178
6925
160
7484
222
77831
132
77960
112
80031
368
80653
135
119995
406
120134
122
120503
409
121210
407
121559
124
123109
137
123780
119
124117
118
125374
479
125501
297
125718
483
126421
299
126542
481
126916
501
127038
205
127989
388
128115
206
423
Magnesium
HMDB0000547
Magnesium salts are essential in nutrition, being required for the activity of many enzymes, especially those concerned with oxidative phosphorylation. Physiologically, it exists as an ion in the body. It is a component of both intra- and extracellular fluids and is excreted in the urine and feces. Deficiency causes irritability of the nervous system with tetany, vasodilatation, convulsions, tremors, depression, and psychotic behavior. Magnesium ion in large amounts is an ionic laxative, and magnesium sulfate (Epsom salts) is sometimes used for this purpose. So-called "milk of magnesia" is a water suspension of one of the few insoluble magnesium compounds, magnesium hydroxide; the undissolved particles give rise to its appearance and name. Milk of magnesia is a mild base, and is commonly used as an antacid.
22537-22-0
C00305
888
18420
13-HYDROXY-MAGNESIUM-PROTOPORP
865
DB01378
[Mg++]
Mg
InChI=1S/Mg/q+2
JLVVSXFLKOJNIY-UHFFFAOYSA-N
magnesium(2+) ion
24.305
23.985041898
0
magnesium(2+) ion
2
2
FDB003518
Magnesium;Magnesium ions;Magnesium ion;Magnesium, doubly charged positive ion;Magnesium, ion (mg(2+));Mg(2+);Mg2+
PW_C000423
Mg2+
86
8
227
4
268
16
476
2
727
26
811
5
819
18
883
22
936
39
983
3
992
21
1167
46
1483
49
1529
43
1764
14
2124
10
2411
59
2942
23
3126
29
3373
7
4540
31
4774
91
4869
54
4974
56
5253
104
5329
111
5356
112
5376
103
5906
147
5934
151
6038
155
6094
161
6250
166
6484
178
6594
164
6881
160
6979
199
7170
205
7194
206
7227
213
7233
211
7250
214
7310
216
7313
198
7473
222
11763
132
11843
210
12312
225
12324
249
12513
288
12581
226
12729
290
15275
285
15337
308
77137
133
77236
329
77937
336
78393
334
78417
335
78489
115
78522
331
78536
356
78574
130
80020
368
80045
184
80048
372
80623
118
80654
135
80865
15
80965
253
81841
51
93832
383
94900
27
108596
223
110559
390
115687
398
119974
406
120070
122
120247
382
120702
407
120981
408
121181
124
121265
429
121319
419
121924
125
122086
405
122408
422
122759
120
122921
399
123307
119
123546
374
123835
464
123889
455
124477
136
124637
376
124978
375
125447
297
125598
484
125669
479
125777
481
125921
482
125947
299
125973
495
126000
490
126243
478
126553
491
126753
300
127125
389
127164
501
127380
502
127407
388
127451
507
127804
209
128125
508
128347
395
1420
Water
HMDB0002111
Water is a chemical substance that is essential to all known forms of life. It appears colorless to the naked eye in small quantities, though it is actually slightly blue in color. It covers 71% of Earth's surface. Current estimates suggest that there are 1.4 billion cubic kilometers (330 million m3) of it available on Earth, and it exists in many forms. It appears mostly in the oceans (saltwater) and polar ice caps, but it is also present as clouds, rain water, rivers, freshwater aquifers, lakes, and sea ice. Water in these bodies perpetually moves through a cycle of evaporation, precipitation, and runoff to the sea. Clean water is essential to human life. In many parts of the world, it is in short supply. From a biological standpoint, water has many distinct properties that are critical for the proliferation of life that set it apart from other substances. It carries out this role by allowing organic compounds to react in ways that ultimately allow replication. All known forms of life depend on water. Water is vital both as a solvent in which many of the body's solutes dissolve and as an essential part of many metabolic processes within the body. Metabolism is the sum total of anabolism and catabolism. In anabolism, water is removed from molecules (through energy requiring enzymatic chemical reactions) in order to grow larger molecules (e.g. starches, triglycerides and proteins for storage of fuels and information). In catabolism, water is used to break bonds in order to generate smaller molecules (e.g. glucose, fatty acids and amino acids to be used for fuels for energy use or other purposes). Water is thus essential and central to these metabolic processes. Water is also central to photosynthesis and respiration. Photosynthetic cells use the sun's energy to split off water's hydrogen from oxygen. Hydrogen is combined with CO2 (absorbed from air or water) to form glucose and release oxygen. All living cells use such fuels and oxidize the hydrogen and carbon to capture the sun's energy and reform water and CO2 in the process (cellular respiration). Water is also central to acid-base neutrality and enzyme function. An acid, a hydrogen ion (H+, that is, a proton) donor, can be neutralized by a base, a proton acceptor such as hydroxide ion (OH-) to form water. Water is considered to be neutral, with a pH (the negative log of the hydrogen ion concentration) of 7. Acids have pH values less than 7 while bases have values greater than 7. Stomach acid (HCl) is useful to digestion. However, its corrosive effect on the esophagus during reflux can temporarily be neutralized by ingestion of a base such as aluminum hydroxide to produce the neutral molecules water and the salt aluminum chloride. Human biochemistry that involves enzymes usually performs optimally around a biologically neutral pH of 7.4. (Wikipedia).
7732-18-5
C00001
962
15377
937
O
H2O
InChI=1S/H2O/h1H2
XLYOFNOQVPJJNP-UHFFFAOYSA-N
water
18.0153
18.010564686
1
water
0
0
FDB013390
Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;Wasser
PW_C001420
H2O
55
8
94
9
109
5
139
4
151
3
162
14
481
13
526
15
624
28
652
10
691
20
770
33
823
18
838
2
1094
31
1377
49
1465
54
1590
43
2018
24
2532
22
2678
60
2727
46
2778
17
2805
29
3143
70
3164
72
3634
61
4598
36
4727
37
4941
93
5030
27
5156
7
5195
97
5214
100
5227
94
5236
103
5297
105
5319
111
5343
113
5355
112
5402
110
5470
123
5483
125
5492
126
5507
127
5534
130
5537
114
5541
129
5591
135
5608
118
5622
108
5691
6
5759
140
5778
101
5841
143
5853
146
5877
107
5890
95
5910
147
5940
151
6032
155
6059
157
6087
161
6123
163
6133
159
6215
1
6218
166
6477
178
6507
180
6600
152
6713
117
6840
188
6888
160
7162
205
7181
207
7193
206
7211
211
7228
213
7238
214
7243
215
7295
198
7350
216
7388
210
7401
212
7467
222
7492
224
7500
190
7588
170
8201
225
8237
226
8414
162
9265
26
11850
277
11922
164
12011
281
12213
285
12250
286
12264
287
12327
249
12520
227
12632
65
12693
290
12705
291
12715
292
13007
298
13019
300
13025
301
13037
302
13261
223
13327
294
15340
308
42327
315
42695
318
43691
322
76914
293
77019
253
77102
132
77131
133
77215
134
77378
331
77397
332
77471
333
77516
115
77536
334
77628
336
77722
337
77759
341
77816
343
77982
347
78071
329
78235
352
78242
353
78270
356
79113
360
80014
368
80039
370
80591
228
80656
119
93830
383
94794
384
110557
390
110639
391
115844
398
119879
232
119915
122
119963
406
120008
407
120046
408
120113
124
120365
412
120430
405
120438
409
120606
415
120794
414
121158
425
121240
429
121351
121
121381
419
121607
434
122118
382
122384
436
122753
120
122797
374
122804
443
123012
446
123064
376
123072
137
123131
447
123142
136
123162
448
123231
451
123384
450
123730
460
123810
464
123940
455
124165
469
124670
399
124938
471
124945
472
125305
297
125353
479
125386
481
125424
482
125480
299
125682
483
125707
478
125745
487
126054
490
126238
495
126273
484
126764
480
126896
501
126963
502
127017
388
127177
208
127199
209
127227
504
127506
507
127576
515
127836
389
128082
395
128176
513
79
Fructose 6-phosphate
HMDB0000124
Fructose 6-phosphate is an important intermediate in the Carbohydrates pathway. The interconversion of glucose-6-phosphate and fructose-6-phosphate, the second step of the Embden-Meyerhof glycolytic pathway, is catalyzed by the enzyme phosphoglucose isomerase (PGI). In gluconeogenesis, fructose-6-phosphate is the immediate precursor of glucose-6-phosphate (wikipedia).
643-13-0
C00085
69507
15946
FRUCTOSE-6P
62713
OCC(=O)[C@@H](O)[C@H](O)[C@H](O)COP(O)(O)=O
C6H13O9P
InChI=1S/C6H13O9P/c7-1-3(8)5(10)6(11)4(9)2-15-16(12,13)14/h4-7,9-11H,1-2H2,(H2,12,13,14)/t4-,5-,6-/m1/s1
GSXOAOHZAIYLCY-HSUXUTPPSA-N
{[(2R,3R,4S)-2,3,4,6-tetrahydroxy-5-oxohexyl]oxy}phosphonic acid
260.1358
260.029718526
-1.05
6
D-fructose 6-phosphate
0
-2
FDB021896
D-fructose 6-phosphate;D-fructose 6-phosphorate;D-fructose 6-phosphoric acid;D-fructose-6-p;D-fructose-6-phosphate;Fpc;Fru-6-p;Fructose 6-phosphate;Fructose-6-p;Fructose-6-phosphate;Fructose-6p;Neuberg ester
PW_C000079
Fru6P
388
8
1014
3
1785
2
5904
147
5932
151
6465
108
6878
160
12987
166
13297
225
42676
315
77093
132
77294
111
77919
112
80660
135
120142
122
120716
407
121178
124
123321
119
123750
118
125504
297
125944
299
127041
205
127404
388
969
Glucosamine 6-phosphate
HMDB0001254
Glucosamine 6-phosphate is normally produced in endothelial cells via the de novo glucosamine synthesis by the enzyme fructose-6-phosphate amidotransferase and the modulation of this pathway by hyperglycemia and glutamine. glutamine-fructose-6-phosphate amidotransferase (GFAT) catalyzes the first committed step in the pathway for biosynthesis of hexosamines in mammals. A member of the N-terminal nucleophile class of amidotransferases, GFAT transfers the amino group from the L-glutamine amide to D-fructose 6-phosphate, producing glutamic acid and glucosamine 6-phosphate. As glucosamine inhibits endothelial nitric oxide synthesis it has important implications for impaired endothelium-dependent relaxation and vascular dysfunction in diabetes mellitus. (PMID 11270676, 11842094).
3616-42-0
C00352
439217
15873
D-GLUCOSAMINE-6-P
388356
N[C@H]1[C@@H](O)O[C@H](COP(O)(O)=O)[C@@H](O)[C@@H]1O
C6H14NO8P
InChI=1S/C6H14NO8P/c7-3-5(9)4(8)2(15-6(3)10)1-14-16(11,12)13/h2-6,8-10H,1,7H2,(H2,11,12,13)/t2-,3-,4-,5-,6+/m1/s1
XHMJOUIAFHJHBW-UKFBFLRUSA-N
{[(2R,3S,4R,5R,6S)-5-amino-3,4,6-trihydroxyoxan-2-yl]methoxy}phosphonic acid
259.151
259.045702941
-0.87
6
α-D-glucosamine 6-phosphate
0
-1
FDB022515
2-amino-2-deoxy-d-glucose 6-phosphate;2-amino-2-deoxyglucose 6-phosphate;2-amino-d-glucose-6-phosphate;D-glucosamine 6-phosphate;D-glucosamine phosphate;D-glucosamine-6-phosphate;Glucosamine 6 -phosphate;Glucosamine 6-phosphate;Glucosamine-6-p;Glucosamine-6-phosphate;Glucose-6-phosphorate;Glucose-6-phosphoric acid;Phosphoric acid mono-((2r,3s,4r,5r)-5-amino-2,3,4-trihydroxy-6-oxo-hexyl) ester;2-amino-2-deoxy-6-o-phosphono-alpha-d-glucopyranose;Alpha-d-glucosamine 6-(dihydrogen phosphate);2-amino-2-deoxy-6-o-phosphono-a-d-glucopyranose;2-amino-2-deoxy-6-o-phosphono-α-d-glucopyranose
PW_C000969
G6P
383
8
6159
108
6224
166
42703
315
77288
111
80661
135
120143
122
125505
297
127042
205
940
Acetyl-CoA
HMDB0001206
The main function of coenzyme A is to carry acyl groups (such as the acetyl group) or thioesters. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA, which is a vital component in cholesterol and ketone synthesis. (wikipedia). acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.
72-89-9
C00024
444493
15351
ACETYL-COA
392413
CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N
C23H38N7O17P3S
InChI=1S/C23H38N7O17P3S/c1-12(31)51-7-6-25-14(32)4-5-26-21(35)18(34)23(2,3)9-44-50(41,42)47-49(39,40)43-8-13-17(46-48(36,37)38)16(33)22(45-13)30-11-29-15-19(24)27-10-28-20(15)30/h10-11,13,16-18,22,33-34H,4-9H2,1-3H3,(H,25,32)(H,26,35)(H,39,40)(H,41,42)(H2,24,27,28)(H2,36,37,38)/t13-,16-,17-,18+,22-/m1/s1
ZSLZBFCDCINBPY-ZSJPKINUSA-N
{[(2R,3S,4R,5R)-2-({[({[(3R)-3-[(2-{[2-(acetylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]-3-hydroxy-2,2-dimethylpropoxy](hydroxy)phosphoryl}oxy)(hydroxy)phosphoryl]oxy}methyl)-5-(6-amino-9H-purin-9-yl)-4-hydroxyoxolan-3-yl]oxy}phosphonic acid
809.571
809.125773051
-2.27
9
acetyl-CoA
0
-4
FDB022491
Ac-coa;Ac-coenzyme a;Ac-s-coa;Ac-s-coenzyme a;Acetyl coenzyme-a;Acetyl-coa;Acetyl-coenzyme a;Acetyl-s-coa;Acetyl-s-coenzyme a;Acetylcoenzyme-a;S-acetate coa;S-acetate coenzyme a;S-acetyl coenzyme a;Accoa;Acetyl coenzyme a;S-acetyl-coa;S-acetyl-coenzyme a;Acetylcoenzyme a
PW_C000940
Ac-CoA
213
4
385
8
842
3
2416
2
2446
5
2896
17
3340
11
4840
14
5278
103
5476
124
5733
108
6025
155
6077
161
6386
1
6470
178
6923
160
7106
163
7291
198
7460
222
8245
151
8277
210
12582
226
13012
299
42615
315
77121
133
77291
111
77562
112
77706
132
77994
115
78355
134
78433
334
80007
368
80634
119
80663
376
90124
170
119953
406
120145
405
120304
122
120632
407
122417
408
122626
384
122743
120
122959
135
123137
118
124986
374
125200
121
125343
479
125507
478
125633
297
126564
482
126572
481
126778
480
126886
501
127044
209
127394
205
127665
388
128137
502
128145
206
128374
391
837
N-Acetyl-D-Glucosamine 6-Phosphate
HMDB0001062
N-Acetyl-D-glucosamine 6-phosphate, also known as N-acetyl-D-glucosamine-6-p or GLCNAC6P, belongs to the class of organic compounds known as acylaminosugars. These are organic compounds containing a sugar linked to a chain through N-acyl group. N-Acetyl-D-glucosamine 6-phosphate is soluble (in water) and a moderately acidic compound (based on its pKa). N-Acetyl-D-glucosamine 6-phosphate has been primarily detected in saliva. Within the cell, N-acetyl-D-glucosamine 6-phosphate is primarily located in the cytoplasm. In humans, N-acetyl-D-glucosamine 6-phosphate is involved in the amino sugar metabolism pathway, the homocarnosinosis pathway, and the glutamate metabolism pathway. N-Acetyl-D-glucosamine 6-phosphate is also involved in several metabolic disorders, some of which include the 2-hydroxyglutric aciduria (D and L form) pathway, the tay-sachs disease pathway, the hyperinsulinism-hyperammonemia syndrome pathway, and the sialuria or french type sialuria pathway. N-Acetyl-D-Glucosamine 6-Phosphate is an intermediate in the metabolism of Aminosugars. It is a substrate for Glucosamine 6-phosphate N-acetyltransferase.
102029-88-9
C00357
440996
15784
N-ACETYL-D-GLUCOSAMINE-6-P
389821
CC(=O)N[C@H]1C(O)O[C@H](COP(O)(O)=O)[C@@H](O)[C@@H]1O
C8H16NO9P
InChI=1S/C8H16NO9P/c1-3(10)9-5-7(12)6(11)4(18-8(5)13)2-17-19(14,15)16/h4-8,11-13H,2H2,1H3,(H,9,10)(H2,14,15,16)/t4-,5-,6-,7-,8?/m1/s1
BRGMHAYQAZFZDJ-RTRLPJTCSA-N
{[(2R,3S,4R,5R)-5-acetamido-3,4,6-trihydroxyoxan-2-yl]methoxy}phosphonic acid
301.1877
301.056267627
-1.23
6
[(2R,3S,4R,5R)-5-acetamido-3,4,6-trihydroxyoxan-2-yl]methoxyphosphonic acid
0
-2
FDB022401
N-acetyl-d-glucosamine 6-phosphate;N-acetyl-d-glucosamine-6-p;N-acetyl-d-glucosamine-6-phosphate;N-acetyl-glucosamine-6-p;N-acetyl-glucosamine-6-phosphate;N-acetylglucosamine-6-p;2-acetamido-2-deoxy-6-o-phosphono-d-glucopyranose;N-acetyl-d-glucosamine 6-phosphoric acid
PW_C000837
NAcG6P
381
8
4841
14
6157
108
6222
166
42389
315
77286
111
77995
115
80664
376
120146
405
120300
122
122957
135
125508
478
125631
297
127045
209
1099
Coenzyme A
HMDB0001423
Coenzyme A (CoA, CoASH, or HSCoA) is a coenzyme notable for its role in the synthesis and oxidization of fatty acids and the oxidation of pyruvate in the citric acid cycle. It is adapted from beta-mercaptoethylamine, panthothenate, and adenosine triphosphate. It is also a parent compound for other transformation products, including but not limited to, phenylglyoxylyl-CoA, tetracosanoyl-CoA, and 6-hydroxyhex-3-enoyl-CoA. Coenzyme A is synthesized in a five-step process from pantothenate and cysteine. In the first step pantothenate (vitamin B5) is phosphorylated to 4'-phosphopantothenate by the enzyme pantothenate kinase (PanK, CoaA, CoaX). In the second step, a cysteine is added to 4'-phosphopantothenate by the enzyme phosphopantothenoylcysteine synthetase (PPC-DC, CoaB) to form 4'-phospho-N-pantothenoylcysteine (PPC). In the third step, PPC is decarboxylated to 4'-phosphopantetheine by phosphopantothenoylcysteine decarboxylase (CoaC). In the fourth step, 4'-phosphopantetheine is adenylylated to form dephospho-CoA by the enzyme phosphopantetheine adenylyl transferase (CoaD). Finally, dephospho-CoA is phosphorylated using ATP to coenzyme A by the enzyme dephosphocoenzyme A kinase (CoaE). Since coenzyme A is, in chemical terms, a thiol, it can react with carboxylic acids to form thioesters, thus functioning as an acyl group carrier. CoA assists in transferring fatty acids from the cytoplasm to the mitochondria. A molecule of coenzyme A carrying an acetyl group is also referred to as acetyl-CoA. When it is not attached to an acyl group, it is usually referred to as 'CoASH' or 'HSCoA'. Coenzyme A is also the source of the phosphopantetheine group that is added as a prosthetic group to proteins such as acyl carrier proteins and formyltetrahydrofolate dehydrogenase. Acetyl-CoA is an important molecule itself. It is the precursor to HMG CoA which is a vital component in cholesterol and ketone synthesis. Furthermore, it contributes an acetyl group to choline to produce acetylcholine in a reaction catalysed by choline acetyltransferase. Its main task is conveying the carbon atoms within the acetyl group to the citric acid cycle to be oxidized for energy production (Wikipedia).
85-61-0
C00010
6816
1146900
CO-A
6557
CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCS
C21H36N7O16P3S
InChI=1S/C21H36N7O16P3S/c1-21(2,16(31)19(32)24-4-3-12(29)23-5-6-48)8-41-47(38,39)44-46(36,37)40-7-11-15(43-45(33,34)35)14(30)20(42-11)28-10-27-13-17(22)25-9-26-18(13)28/h9-11,14-16,20,30-31,48H,3-8H2,1-2H3,(H,23,29)(H,24,32)(H,36,37)(H,38,39)(H2,22,25,26)(H2,33,34,35)/t11-,14-,15-,16+,20-/m1/s1
RGJOEKWQDUBAIZ-IBOSZNHHSA-N
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-({2-[(2-sulfanylethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
767.534
767.115208365
-2.22
10
coenzyme A
0
-4
FDB022614
Acetoacetyl coenzyme a sodium salt;Coa;Coa hydrate;Coa-sh;Coash;Coenzyme a;Coenzyme a hydrate;Coenzyme a-sh;Coenzyme ash;Coenzymes a;Depot-zeel;Propionyl coa;Propionyl coenzyme a;S-propanoate;S-propanoate coa;S-propanoate coenzyme a;S-propanoic acid;S-propionate coa;S-propionate coenzyme a;Zeel;[(2r,3s,4r,5r)-5-(6-amino-9h-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydrofuran-2-yl]methyl 3-hydroxy-4-({3-oxo-3-[(2-sulfanylethyl)amino]propyl}amino)-2,2-dimethyl-4-oxobutyl dihydrogen diphosphate
PW_C001099
CoA
211
4
386
8
845
3
879
22
892
17
2407
59
2414
2
2459
5
2813
29
2862
31
3342
11
3351
18
4618
10
4629
58
4842
14
4865
54
4879
6
5232
102
5247
104
5280
103
5477
124
5734
108
5777
101
6023
155
6075
161
6384
1
6468
178
6930
160
6961
162
6973
199
7083
188
7108
163
7293
198
7347
210
7458
222
8229
151
9081
226
9090
224
9124
170
9215
195
13013
299
15318
249
25488
49
42616
315
76907
293
77119
133
77222
134
77230
329
77292
111
77550
132
77555
334
77563
112
77633
336
77672
129
77996
115
78047
332
78056
350
78413
335
78567
130
79259
333
79974
331
80005
368
80620
118
80627
374
80635
119
80665
376
93828
382
93834
383
98674
288
110555
389
110561
390
115842
399
115847
398
119951
406
120147
405
120231
384
120305
122
120634
407
120762
117
121406
123
121421
433
121521
125
121666
429
121682
408
121714
414
122404
422
122741
120
122904
121
122960
135
123965
447
123979
468
124079
136
124220
464
124265
450
124974
375
125341
479
125509
478
125579
480
125592
484
125634
297
126084
481
126549
491
126560
482
126746
300
126884
501
127046
209
127109
391
127301
205
127540
206
127667
388
128121
508
128133
502
128340
395
914
Adenosine diphosphate ribose
HMDB0001178
Adenosine diphosphate ribose is a molecule formed into poly(ADP-ribose) or PAR chains by the enzyme poly ADP ribose polymerase or PARP. PARP is found in every cell nucleus. Its main role is to detect and signal single-strand DNA breaks (SSB) to the enzymatic machinery involved in the SSB repair. PARP activation is an immediate cellular response to metabolic, chemical, or radiation-induced DNA SSB damage. Once PARP detects a SSB, it binds to the DNA, and, after a structural change, begins the synthesis of a poly (ADP-ribose) chain (PAR) as a signal for the other DNA-repairing enzymes such as DNA ligase III (LigIII), DNA polymerase beta, and scaffolding proteins such as X-ray cross-complementing gene 1 (XRCC1). After repairing, the PAR chains are degraded via PAR glycohydrolase (PARG). ADP-ribose binds to and activates the TRPM2 ion channel. Adenosine diphosphate ribose is an intermediate in NAD metabolism. The enzyme NAD(P)+ nucleosidase [EC:3.2.2.6] catalyzes the production of this metabolite from nicotinamide adenine dinucleotide phosphate. This reaction is irreversible and occurs in the cytosol.
20762-30-5
C00301
192
16960
ADENOSINE_DIPHOSPHATE_RIBOSE
187
NC1=C2N=CN(C3OC(COP(O)(=O)OP(O)(=O)OCC4OC(O)C(O)C4O)C(O)C3O)C2=NC=N1
C15H23N5O14P2
InChI=1S/C15H23N5O14P2/c16-12-7-13(18-3-17-12)20(4-19-7)14-10(23)8(21)5(32-14)1-30-35(26,27)34-36(28,29)31-2-6-9(22)11(24)15(25)33-6/h3-6,8-11,14-15,21-25H,1-2H2,(H,26,27)(H,28,29)(H2,16,17,18)
SRNWOUGRCWSEMX-UHFFFAOYSA-N
{[5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}({hydroxy[(3,4,5-trihydroxyoxolan-2-yl)methoxy]phosphoryl}oxy)phosphinic acid
559.3157
559.071673493
-2.19
8
adp ribose
0
-2
FDB022468
(rib5)ppa;5-(adenosine 5'-pyrophosphoryl)-d-ribose;A5'pp5rib;Adp ribose;Adp-d-ribose;Adp-rib;Adp-ribose;Adpribose;Adenosine 5'-diphosphoribose;Adenosine diphosphate ribose;Adopprib;Ribose adenosine diphosphate;[5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl [hydroxy-[(3,4,5-trihydroxyoxolan-2-yl)methoxy]phosphoryl] hydrogen phosphate;D-ribofuranos-5-yl-adp
PW_C000914
ADP-Rib
1837
8
4571
2
11788
198
77997
111
78391
132
80667
135
120149
122
121952
124
124505
118
125511
297
127048
205
127710
388
1858
Chitin
HMDB0003362
Chitin is one of the main components in the cell walls of fungi, the exoskeletons of insects and other arthropods (such as crustaceans) as well as fish and frogs. It is a polysaccharide that is constructed from units of acetylglucosamine (more completely, N-acetyl-D-glucose-2-amine). These are linked together in beta-1,4 fashion (in a similar manner to the glucose units which form cellulose). In effect, chitin may be described as cellulose with one hydroxyl group on each monomer replaced by an acetylamine group. This allows for increased hydrogen bonding between adjacent polymers, giving the polymer increased strength. Chitin is the second most abundant polysaccharide in the world (after cellulose). Chitinases break down chitin and are a part of the defence mechanism of mammals against chitin-containing parasites in lower life forms. Under certain circumstances, chitin can act as an allergen. Research using murine models has shown that chitin is a size-dependent microbial-associated molecular pattern (MAMP) that can induce an immunological response via pattern recognition receptors. Medium-sized chitin micro-particles (CMPs) have been shown to induce inflammation, while small-sized CMPs reduce inflammation. Additionally, mammalian chitinases may play a key role in mediating the T-helper 2 cell-driven inflammatory response that is commonly associated with asthma. The high prevalence of asthma among people working with chitinous substances, such as crabs and fungi, suggests that chitin might be an allergen playing a significant role in the development of asthma.
1398-61-4
C00461
21252321
17029
14-N-ACETYL-BETA-D-GLUCOSAMINYLN1
399508
CC(=O)N[C@H]1[C@H](O)O[C@H](CO)[C@@H](O[C@@H]2O[C@H](CO)[C@@H](O[C@@H]3O[C@H](CO)[C@@H](O)[C@H](O)[C@H]3NC(C)=O)[C@H](O)[C@H]2NC(C)=O)[C@@H]1O
C24H41N3O16
InChI=1S/C24H41N3O16/c1-7(31)25-13-18(36)20(11(5-29)39-22(13)38)42-24-15(27-9(3)33)19(37)21(12(6-30)41-24)43-23-14(26-8(2)32)17(35)16(34)10(4-28)40-23/h10-24,28-30,34-38H,4-6H2,1-3H3,(H,25,31)(H,26,32)(H,27,33)/t10-,11-,12-,13-,14-,15-,16-,17-,18-,19-,20-,21-,22-,23+,24+/m1/s1
WZZVUHWLNMNWLW-VFCSDQTKSA-N
627.5928
627.248682279
-0.92
0
FDB023154
Poly 2-acetamido-2-deoxy-d-glucose;Poly 2-acetamido-2-deoxy-delta-glucose;[1,4-(n-acetyl-beta-d-glucosaminyl)]n;[1,4-(n-acetyl-beta-d-glucosaminyl)]n+1;[1,4-(n-acetyl-beta-delta-glucosaminyl)]n;[1,4-(n-acetyl-beta-delta-glucosaminyl)]n+1;Beta-1,4-poly-n-acetyl-d-glucosamine;Beta-1,4-poly-n-acetyl-delta-glucosamine
PW_C001858
Chitin
376
9
4853
8
6167
108
6234
166
42707
315
77281
113
77998
111
80668
135
120150
122
120293
126
122952
443
125512
297
125626
301
127049
205
1916
Carbonic acid
HMDB0003538
Carbonic acid (ancient name acid of air or aerial acid) is the only inorganic carbon acid, and has the formula H2CO3. It is also a name sometimes given to solutions of carbon dioxide in water, which contain small amounts of H2CO3. The salts of carbonic acids are called bicarbonates (or hydrogencarbonates) and carbonates. (wikipedia).
463-79-6
C01353
22639876
28976
CARBON-DIOXIDE
747
OC(O)=O
CH2O3
InChI=1S/CH2O3/c2-1(3)4/h(H2,2,3,4)
BVKZGUZCCUSVTD-UHFFFAOYSA-N
carbonic acid
62.0248
62.00039393
0.57
2
carbonic acid
0
-1
FDB023191
Acid of air;Aerial acid;Bisodium carbonate;Calcined;Carbonic acid sodium salt;Consal;Crystol carbonate;Dihydrogen carbonate;Disodium carbonate;Mild alkali;Na-x;Oxyper;Sal soda;Salt of soda;Scotch soda;Soda;Soda ash;Sodium carbonate;Sodium carbonate anhydrous;Sodium carbonate hydrated;Sodium carbonate peroxyhydrate;Solvay soda;Trona soda ash;Tronalight light soda ash;[co(oh)2];H2co3;Koehlensaeure;Carbonate;Dihydrogen carbonic acid
PW_C001916
CarbncA
4854
8
11902
170
77999
111
80670
135
120152
122
125514
297
127051
205
1441
Phosphoric acid
HMDB0002142
Phosphoric acid, also known as orthophosphoric acid or phosphoric(V) acid, is a mineral acid with the chemical formula H3PO4. Alternatively, orthophosphoric acid molecules can combine with themselves to form a variety of compounds referred to as phosphoric acids in a more general way. For a discussion of these, see Phosphoric acids and Phosphates. Appears to exist only as a food additive and produced synthetically. --Wikipedia.
7664-38-2
C00009
1004
26078
979
OP(O)(O)=O
H3O4P
InChI=1S/H3O4P/c1-5(2,3)4/h(H3,1,2,3,4)
NBIIXXVUZAFLBC-UHFFFAOYSA-N
phosphoric acid
97.9952
97.976895096
3
phosphoric acid
0
-2
FDB013380
Acide phosphorique (french);Acido fosforico [italian];Acidum phosphoricum;Diphosphate tetrasodium;Fosforzuuroplossingen [dutch];Marphos;Nfb;Ortho- phosphoric acid;Orthophosphoric acid;Phosphoric acid (acd/name 4.0);Phosphorsaeure;Phosphorsaeureloesungen [german];Sodium pyrophosphate;Sodium pyrophosphate decahydrate;Sodium pyrophosphate decahydrate biochemica;Sonac;Tetra-sodium pyrophosphate;Tetrasodium pyrophosphate decahydrate;Tetrasodium pyrophosphate 10-hydrate;White phosphoric acid;[po(oh)3];Acide phosphorique;H3po4;Phosphate;Phosphorsaeureloesungen;Orthophosphate
PW_C001441
PhosfrA
171
8
440
4
483
13
1592
43
1678
2
1902
49
1910
5
4856
3
5913
147
5943
151
6891
160
11782
198
11828
213
11829
216
11852
277
12638
31
77105
132
77330
133
77473
333
77779
111
78000
112
78272
356
78706
331
78707
334
80671
135
80677
119
119918
122
120126
124
120157
407
120344
406
120367
412
121383
419
121900
383
121902
408
122858
118
122994
120
123014
446
123942
455
124453
398
124455
374
125308
297
125493
299
125518
481
125664
479
126056
490
126855
205
127030
388
127053
206
127159
501
127508
507
862
Carbamoyl phosphate
HMDB0001096
Carbamoyl phosphate is a precursor of both arginine and pyrimidine biosynthesis. It is a labile and potentially toxic intermediate. Carbamoyl phosphate is a molecule that is involved in ridding the body of excess nitrogen in the urea cycle, and also in the synthesis of pyrimidines. It is produced from carbon dioxide, ammonia, and phosphate (from ATP) by the enzyme carbamoyl phosphate synthase. -- Wikipedia.
590-55-6
C00169
278
17672
CARBAMOYL-P
272
NC(=O)OP(O)(O)=O
CH4NO5P
InChI=1S/CH4NO5P/c2-1(3)7-8(4,5)6/h(H2,2,3)(H2,4,5,6)
FFQKYPRQEYGKAF-UHFFFAOYSA-N
(carbamoyloxy)phosphonic acid
141.0199
140.982708755
-0.96
3
carbamoyl-phosphate
0
-2
FDB022424
Carbamic acid monoanhydride with phosphorate;Carbamic acid monoanhydride with phosphoric acid;Carbamoyl-p;Carbamoyl-phosphate;Carbamoylphosphate;Carbamyl phosphate;Carbamyl-phosphate;Aminocarbonyl dihydrogen phosphate;Carbamic phosphoric monoanhydride;Carbamoyl phosphate;Monocarbamoyl phosphate;Phosphoric acid mono(formamide)ester;Aminocarbonyl dihydrogen phosphoric acid;Carbamoyl phosphoric acid;Carbamoyl phosphoric acid;Carbamyl phosphoric acid;Monocarbamoyl phosphoric acid;Phosphate mono(formamide)ester
PW_C000862
CarbmP
89
8
441
4
2702
2
9268
13
11853
161
12730
290
77331
133
77547
111
78725
132
80672
135
120092
122
120345
406
122199
124
122995
120
124751
118
125462
297
125665
479
126361
299
126999
205
127160
501
127923
388
1005
Zinc (II) ion
HMDB0001303
Zinc is an essential element, necessary for sustaining all life.Physiologically, it exists as an ion in the body. It is estimated that 3000 of the hundreds of thousands of proteins in the human body contain zinc prosthetic groups. In addition, there are over a dozen types of cells in the human body that secrete zinc ions, and the roles of these secreted zinc signals in medicine and health are now being actively studied. Intriguingly, brain cells in the mammalian forebrain are one type of cell that secretes zinc, along with its other neuronal messenger substances. Cells in the salivary gland, prostate, immune system and intestine are other types that secrete zinc. Obtaining a sufficient zinc intake during pregnancy and in young children is a problem, especially among those who cannot afford a good and varied diet. Brain development is stunted by zinc deficiency in utero and in youth. Zinc is an activator of certain enzymes, such as carbonic anhydrase. Carbonic anhydrase is important in the transport of carbon dioxide in vertebrate blood. Even though zinc is an essential requirement for a healthy body, too much zinc can be harmful. Excessive absorption of zinc can also suppress copper and iron absorption. The free zinc ion in solution is highly toxic to plants, invertebrates, and even vertebrate fish. The Free Ion Activity Model (FIAM) is well-established in the literature, and shows that just micromolar amounts of the free ion kills some organisms.
23713-49-7
C00038
32051
29105
ZN%2b2
29723
DB01593
[Zn++]
Zn
InChI=1S/Zn/q+2
PTFCDOFLOPIGGS-UHFFFAOYSA-N
zinc(2+) ion
65.409
63.929146578
0
zinc(2+) ion
2
2
FDB003729
Zinc;Zinc ion;Dietary zinc;Zinc cation;Zinc, ion (zn2+);Zn(ii);Zn(2+);Zn2+
PW_C001005
Zinc
13
2
38
4
118
8
271
16
529
15
2957
51
3044
68
3120
29
3147
70
5410
117
5425
103
5434
118
5459
120
5560
132
5585
133
5598
135
7449
166
11787
198
12466
226
12724
290
13321
151
76967
225
77401
111
77580
114
77929
336
80400
1
120020
124
120035
406
120060
122
120441
409
121257
429
123075
137
123827
464
125398
299
125413
479
125438
297
125685
483
126938
388
126953
501
126976
205
127180
208
1316
Carbon dioxide
HMDB0001967
Carbon dioxide is a colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals. Carbon dioxide is produced during respiration by all animals, fungi and microorganisms that depend on living and decaying plants for food, either directly or indirectly. It is, therefore, a major component of the carbon cycle. Additionally, carbon dioxide is used by plants during photosynthesis to make sugars which may either be consumed again in respiration or used as the raw material to produce polysaccharides such as starch and cellulose, proteins and the wide variety of other organic compounds required for plant growth and development. When inhaled at concentrations much higher than usual atmospheric levels, it can produce a sour taste in the mouth and a stinging sensation in the nose and throat. These effects result from the gas dissolving in the mucous membranes and saliva, forming a weak solution of carbonic acid. Carbon dioxide is used by the food industry, the oil industry, and the chemical industry. Carbon dioxide is used to produce carbonated soft drinks and soda water. Traditionally, the carbonation in beer and sparkling wine comes about through natural fermentation, but some manufacturers carbonate these drinks artificially.
124-38-9
C00011
280
16526
274
O=C=O
CO2
InChI=1S/CO2/c2-1-3
CURLTUGMZLYLDI-UHFFFAOYSA-N
methanedione
44.0095
43.989829244
0.63
0
carbon dioxide
0
0
DBMET00423
FDB014084
Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744
PW_C001316
CO2
50
8
121
1
204
4
480
13
503
18
640
3
677
31
695
20
806
5
1133
43
1638
49
1745
2
2551
17
3144
70
5283
103
5320
111
5750
108
5771
101
5968
100
6026
155
6078
161
6471
178
6637
107
6922
190
7017
160
7035
163
7061
188
7163
205
7308
198
7333
213
7461
222
7530
210
8215
225
8223
151
9158
249
11849
277
11908
170
12464
226
12688
290
42626
315
43523
318
76994
293
77122
133
77170
132
77470
333
77739
112
77750
129
77763
341
78077
134
78405
356
78427
334
78941
331
79227
130
80008
368
80675
119
80717
135
94836
384
113291
391
115549
121
119954
406
120089
122
120155
407
120364
412
120556
414
120833
419
120922
124
120991
408
121284
125
121505
383
122744
120
123011
446
123190
450
123418
455
123489
118
123556
374
123855
136
124063
398
125344
479
125460
297
125516
481
125824
490
125870
299
125931
482
126280
480
126887
501
127052
206
127277
507
127331
388
127390
502
882
5-Phosphoribosylamine
HMDB0001128
5-Phosphoribosylamine, also known as 5-p-beta-D-ribosyl-amine or PRA, belongs to the class of organic compounds known as pentose phosphates. These are carbohydrate derivatives containing a pentose substituted by one or more phosphate groups. 5-Phosphoribosylamine is soluble (in water) and a moderately acidic compound (based on its pKa). In humans, 5-phosphoribosylamine is involved in the purine metabolism pathway, the azathioprine action pathway, the mercaptopurine action pathway, and the homocarnosinosis pathway. 5-Phosphoribosylamine is also involved in several metabolic disorders, some of which include the AICA-ribosiduria pathway, xanthine dehydrogenase deficiency (xanthinuria), the hyperinsulinism-hyperammonemia syndrome pathway, and 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency. Outside of the human body, 5-phosphoribosylamine can be found in a number of food items such as red bell pepper, apple, chinese chestnut, and wild leek. This makes 5-phosphoribosylamine a potential biomarker for the consumption of these food products. 5-Phosphoribosylamine is a substrate for Amidophosphoribosyltransferase and Trifunctional purine biosynthetic protein adenosine-3.
C03090
439905
37737
5-P-BETA-D-RIBOSYL-AMINE
388939
N[C@@H]1O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]1O
C5H12NO7P
InChI=1S/C5H12NO7P/c6-5-4(8)3(7)2(13-5)1-12-14(9,10)11/h2-5,7-8H,1,6H2,(H2,9,10,11)/t2-,3-,4-,5-/m1/s1
SKCBPEVYGOQGJN-TXICZTDVSA-N
{[(2R,3S,4R,5R)-5-amino-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid
229.125
229.035138255
-0.78
5
5-phospho-β-D-ribosylamine
0
-1
FDB022441
5-p-beta-d-ribosyl-amine;5-p-beta-delta-ribosyl-amine;5-phospho-d-ribosylamine;5-phospho-beta-d-ribosyl-amine;5-phospho-beta-d-ribosylamine;5-phospho-beta-delta-ribosyl-amine;5-phospho-beta-delta-ribosylamine;5-phospho-delta-ribosylamine;5-phosphoribosylamine;Pra;5-phosphoribosyl-1-amine;5-phospho-b-d-ribosylamine;5-phospho-β-d-ribosylamine
PW_C000882
5-PPRSA
1841
8
11791
198
78001
111
80679
135
120159
122
125520
297
127055
205
170
Pyrophosphate
HMDB0000250
The anion, the salts, and the esters of pyrophosphoric acid are called pyrophosphates. The pyrophosphate anion is abbreviated PPi and is formed by the hydrolysis of ATP into AMP in cells. This hydrolysis is called pyrophosphorolysis. The pyrophosphate anion has the structure P2O74-, and is an acid anhydride of phosphate. It is unstable in aqueous solution and rapidly hydrolyzes into inorganic phosphate. Pyrophosphate is an osteotoxin (arrests bone development) and an arthritogen (promotes arthritis). It is also a metabotoxin (an endogenously produced metabolite that causes adverse health affects at chronically high levels). Chronically high levels of pyrophosphate are associated with hypophosphatasia. Hypophosphatasia (also called deficiency of alkaline phosphatase or phosphoethanolaminuria) is a rare, and sometimes fatal, metabolic bone disease. Hypophosphatasia is associated with a molecular defect in the gene encoding tissue non-specific alkaline phosphatase (TNSALP). TNSALP is an enzyme that is tethered to the outer surface of osteoblasts and chondrocytes. TNSALP hydrolyzes several substances, including inorganic pyrophosphate (PPi) and pyridoxal 5'-phosphate (PLP), a major form of vitamin B6. When TSNALP is low, inorganic pyrophosphate (PPi) accumulates outside of cells and inhibits the formation of hydroxyapatite, one of the main components of bone, causing rickets in infants and children and osteomalacia (soft bones) in adults. Vitamin B6 must be dephosphorylated by TNSALP before it can cross the cell membrane. Vitamin B6 deficiency in the brain impairs synthesis of neurotransmitters which can cause seizures. In some cases, a build-up of calcium pyrophosphate dihydrate crystals in the joints can cause pseudogout.
14000-31-8
C00013
644102
18361
PPI
559142
DB04160
[O-]P([O-])(=O)OP([O-])([O-])=O
O7P2
InChI=1S/H4O7P2/c1-8(2,3)7-9(4,5)6/h(H2,1,2,3)(H2,4,5,6)/p-4
XPPKVPWEQAFLFU-UHFFFAOYSA-J
(phosphonooxy)phosphonic acid
173.9433
173.911925378
4
pyrophosphoric acid
0
-3
FDB021918
(4-)diphosphoric acid ion;(p2o74-)diphosphate;Diphosphate;Diphosphoric acid;Ppi;Pyrometaphosphate;Pyrophosphate;Pyrophosphate tetraanion;Pyrophosphate(4-) ion;[o3popo3](4-);Diphosphat;P2o7(4-);Pyrophosphat;Pyrophosphate ion;Phosphonato phosphoric acid;Pyrophosphoric acid;Pyrophosphoric acid ion
PW_C000170
Ppi
12
2
35
4
63
8
429
23
735
32
882
22
1217
3
1620
49
2410
59
2815
29
4175
14
4868
54
5034
89
5252
104
5294
101
5409
117
5424
103
5433
118
5458
120
5548
111
5559
132
5584
133
5606
135
5655
108
5879
107
6239
166
6978
199
7073
188
7134
163
7272
160
7312
198
7318
213
8275
151
8283
210
11869
161
12002
222
12041
164
12315
225
12323
249
12512
288
12579
226
12695
290
15219
306
15375
18
34760
17
42561
315
42697
318
77235
329
77317
128
77635
336
78416
335
78928
331
79153
112
79950
134
79958
130
80047
372
80417
170
85630
1
94786
384
94814
125
94819
382
98678
223
110634
391
113270
395
113275
389
115527
136
115532
399
119934
122
120017
124
120032
406
120330
410
120936
407
121261
429
121341
121
121486
383
122407
422
122985
444
123502
119
123831
464
124044
398
124977
375
125324
297
125395
299
125410
479
125597
484
125656
485
125876
481
126552
491
126869
205
126935
388
126950
501
127337
206
128124
508
190
Phosphoribosyl pyrophosphate
HMDB0000280
Phosphoribosyl pyrophosphate (PRPP) is a pentosephosphate. The key substance in the biosynthesis of histidine, tryptophan, and purine and pyrimidine nucleotides. It is formed from ribose 5-phosphate by the enzyme ribose-phosphate diphosphokinase. It plays a role in transferring phosphate groups in several reactions. (Wikipedia).
7540-64-9
C00119
7339
17111
PRPP
7062
DB01632
O[C@H]1[C@@H](O)[C@@H](O[P@](O)(=O)OP(O)(O)=O)O[C@@H]1COP(O)(O)=O
C5H13O14P3
InChI=1S/C5H13O14P3/c6-3-2(1-16-20(8,9)10)17-5(4(3)7)18-22(14,15)19-21(11,12)13/h2-7H,1H2,(H,14,15)(H2,8,9,10)(H2,11,12,13)/t2-,3-,4-,5-/m1/s1
PQGCEDQWHSBAJP-TXICZTDVSA-N
[({[(2R,3R,4S,5R)-3,4-dihydroxy-5-[(phosphonooxy)methyl]oxolan-2-yl]oxy}(hydroxy)phosphoryl)oxy]phosphonic acid
390.0696
389.95181466
-1.53
7
phosphoribosylpyrophosphate
0
-4
FDB021928
5-phospho-a-d-ribose-1-diphosphate;5-phospho-a-d-ribosyl pyrophosphate;5-phospho-alpha-d-ribose 1-diphosphate;5-phospho-alpha-d-ribose-1-diphosphate;5-phospho-alpha-d-ribosyl pyrophosphate;5-phospho-alpha-d-riobse 1-diphosphate;5-phospho-alpha-delta-ribose 1-diphosphate;5-phospho-alpha-delta-ribose-1-diphosphate;5-phospho-alpha-delta-ribosyl pyrophosphate;5-phosphoribose 1-pyrophosphate;5-phosphoribosyl 1-diphosphate;5-phosphoribosyl a-1-pyrophosphate;5-phosphoribosyl-1-pyrophosphate;5-phosphorylribose 1-a-diphosphate;5-phosphorylribose 1-alpha-diphosphate;5-phosphorylribose 1-pyrophosphate;5-phosphorylribosyl 1-pyrophosphate;Pp-ribose-p;Prpp;Prib-pp;Phosphoribosyl pyrophosphate;Phosphoribosyl-1-pyrophosphate;Phosphoribosyl-pyrophosphate;Phosphoribosylpyrophosphorate;Phosphoribosylpyrophosphoric acid;A-d-5-(dihydrogen phosphate) 1-(trihydrogen pyrophosphate) ribofuranose;A-d-5-phosphoribosyl 1-pyrophosphate;A-d-ribofuranose 5-phosphate 1-pyrophosphate;A-d-ribofuranose, 5-(dihydrogen phosphate) 1-(trihydrogen diphosphate);Alpha-d-5-(dihydrogen phosphate) 1-(trihydrogen pyrophosphate) ribofuranose;Alpha-d-5-phosphoribosyl 1-pyrophosphate;Alpha-d-ribofuranose 5-phosphate 1-pyrophosphate;Alpha-d-ribofuranose, 5-(dihydrogen phosphate) 1-(trihydrogen diphosphate);Alpha-delta-5-(dihydrogen phosphate) 1-(trihydrogen pyrophosphate) ribofuranose;Alpha-delta-5-phosphoribosyl 1-pyrophosphate;Alpha-delta-ribofuranose 5-phosphate 1-pyrophosphate;5-phosphoribosyl 1-pyrophosphate;5-phosphoribosyl diphosphate;Alpha-d-ribofuranose 5-(dihydrogen phosphate) 1-(trihydrogen diphosphate);Alpha-phosphoribosylpyrophosphoric acid;Phosphoribosylpyrophosphate;5-phospho-a-d-ribose 1-diphosphate;5-phospho-a-d-ribose 1-diphosphoric acid;5-phospho-alpha-d-ribose 1-diphosphoric acid;5-phospho-α-d-ribose 1-diphosphate;5-phospho-α-d-ribose 1-diphosphoric acid;Phosphoribosyl pyrophosphoric acid;5-phosphoribosyl 1-pyrophosphoric acid;5-phosphoribosyl diphosphoric acid;A-d-ribofuranose 5-(dihydrogen phosphate) 1-(trihydrogen diphosphate);A-d-ribofuranose 5-(dihydrogen phosphoric acid) 1-(trihydrogen diphosphoric acid);Alpha-d-ribofuranose 5-(dihydrogen phosphoric acid) 1-(trihydrogen diphosphoric acid);α-d-ribofuranose 5-(dihydrogen phosphate) 1-(trihydrogen diphosphate);α-d-ribofuranose 5-(dihydrogen phosphoric acid) 1-(trihydrogen diphosphoric acid);A-phosphoribosylpyrophosphate;A-phosphoribosylpyrophosphoric acid;Alpha-phosphoribosylpyrophosphate;α-phosphoribosylpyrophosphate;α-phosphoribosylpyrophosphoric acid
PW_C000190
PRPP
1774
2
1815
8
5816
108
11780
198
42663
315
77176
132
78002
111
80680
135
111657
7
120160
122
121950
124
124503
118
125521
297
126346
299
127056
205
127709
388
40711
4Fe-4S
HMDB0061380
Tetrakis(λ¹-iron(1+) ion) tetrasulfane tetrasulfanide belongs to the class of inorganic compounds known as transition metal sulfides. These are inorganic compounds containing a sulfur atom of an oxidation state of -2, in which the heaviest atom bonded to the oxygen is a transition metal.
33723
S[Fe]12[S]3[Fe]4(S)[S]1[Fe]1(S)[S]2[Fe]3(S)[S]41
Fe4H4S8
InChI=1S/4Fe.4H2S.4S/h;;;;4*1H2;;;;/q4*+1;;;;;;;;/p-4
CFWDOBXUEATMSD-UHFFFAOYSA-J
tetrakis(lambda1-iron(1+) ion) tetrasulfane tetrasulfanide
483.932
483.54763418
0
tetrakis(lambda1-iron(1+) ion) tetrasulfane tetrasulfanide
0
1
PW_C040711
4Fe-4S
4738
3
4859
8
5051
4
5065
2
5403
110
6060
157
6124
163
6508
180
7493
224
12618
164
77160
112
78003
111
80040
370
80681
135
117797
133
117828
132
120009
407
120161
122
122658
406
122673
124
122784
119
125233
120
125248
118
125387
481
125522
297
126790
479
126806
299
126928
206
127057
205
128387
501
128404
388
1196
Xanthylic acid
HMDB0001554
Xanthylic acid is an important metabolic intermediate in the Purine Metabolism, and is a product or substrate of the enzymes Inosine monophosphate dehydrogenase (EC 1.1.1.205), Hypoxanthine phosphoribosyltransferase (EC 2.4.2.8), Xanthine phosphoribosyltransferase (EC 2.4.2.22), 5'-Ribonucleotide phosphohydrolase (EC 3.1.3.5), Ap4A hydrolase (EC 3.6.1.17), Nucleoside-triphosphate diphosphatase (EC 3.6.1.19), Phosphoribosylamine-glycine ligase (EC 6.3.4.1), and glutamine amidotransferase (EC 6.3.5.2). (KEGG) Xanthylic acid can also be used in quantitative measurements of the Inosine monophosphate dehydrogenase enzyme activities in purine metabolism. This measurement is important for optimal thiopurine therapy for children with acute lymphoblastic leukaemia (ALL). (PMID: 16725387).
523-98-8
C00655
73323
15652
XANTHOSINE-5-PHOSPHATE
66054
O[C@@H]1[C@@H](COP(O)(O)=O)O[C@H]([C@@H]1O)N1C=NC2=C1NC(=O)NC2=O
C10H13N4O9P
InChI=1S/C10H13N4O9P/c15-5-3(1-22-24(19,20)21)23-9(6(5)16)14-2-11-4-7(14)12-10(18)13-8(4)17/h2-3,5-6,9,15-16H,1H2,(H2,19,20,21)(H2,12,13,17,18)/t3-,5-,6-,9-/m1/s1
DCTLYFZHFGENCW-UUOKFMHZSA-N
{[(2R,3S,4R,5R)-5-(2,6-dioxo-2,3,6,9-tetrahydro-1H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid
364.2054
364.042014546
-2.17
6
xanthosine monophosphate
0
-2
FDB022687
(9-d-ribosylxanthine)-5'-phosphate;5'-xanthonylate monophosphate;Xmp;Xanthosine 5'-phosphate;Xanthosine-5'-p;Xanthosine-5'-phosphate;Xanthosine monophosphate;Xanthylic acid;(9-d-ribosylxanthine)-5'-phosphoric acid;5'-xanthylate;Xanthosine 5'-phosphoric acid;Xanthosine monophosphoric acid;Xanthylate
PW_C001196
XntylcA
1857
8
11808
198
78004
111
80683
135
120164
122
125524
297
127059
205
32
Adenosine monophosphate
HMDB0000045
Adenosine monophosphate, also known as 5'-adenylic acid and abbreviated AMP, is a nucleotide that is found in RNA. It is an ester of phosphoric acid with the nucleoside adenosine. AMP consists of the phosphate group, the pentose sugar ribose, and the nucleobase adenine. AMP can be produced during ATP synthesis by the enzyme adenylate kinase. AMP has recently been approved as a 'Bitter Blocker' additive to foodstuffs. When AMP is added to bitter foods or foods with a bitter aftertaste it makes them seem 'sweeter'. This potentially makes lower calorie food products more palatable.
61-19-8
C00020
6083
16027
AMP
5858
DB00131
NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1
C10H14N5O7P
InChI=1S/C10H14N5O7P/c11-8-5-9(13-2-12-8)15(3-14-5)10-7(17)6(16)4(22-10)1-21-23(18,19)20/h2-4,6-7,10,16-17H,1H2,(H2,11,12,13)(H2,18,19,20)/t4-,6-,7-,10-/m1/s1
UDMBCSSLTHHNCD-KQYNXXCUSA-N
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid
347.2212
347.063084339
-2.02
5
adenylate
0
-2
DBMET00485
FDB021806
5'-amp;5'-adenosine monophosphate;5'-adenylate;5'-adenylic acid;Amp;Adenosine 5'-monophosphate;Adenosine 5'-phosphate;Adenosine 5'-phosphorate;Adenosine 5'-phosphoric acid;Adenosine phosphate;Adenosine-5'-monophosphorate;Adenosine-5'-monophosphoric acid;Adenosine-5-monophosphorate;Adenosine-5-monophosphoric acid;Adenosine-monophosphate;Adenosine-phosphate;Adenovite;Adenylate;Adenylic acid;Cardiomone;Lycedan;Muscle adenylate;Muscle adenylic acid;My-b-den;My-beta-den;Phosaden;Phosphaden;Phosphentaside;5'-o-phosphonoadenosine;Adenosine 5'-(dihydrogen phosphate);Adenosine monophosphate;Adenosine-5'p;Adenosini phosphas;Ado5'p;Fosfato de adenosina;Pa;Pado;Phosphate d'adenosine;5'-adenosine monophosphoric acid;Adenosine phosphoric acid;Adenosine 5'-(dihydrogen phosphoric acid);Adenosine 5'-monophosphoric acid;Adenosine monophosphoric acid;Adenosine-5'-monophosphate;Phosphoric acid d'adenosine
PW_C000032
AMP
11
2
34
4
62
8
270
16
734
32
881
22
1189
14
4572
5
4867
54
5033
89
5251
104
5408
117
5423
103
5432
118
5457
120
5558
132
5583
133
5779
101
5795
108
6977
199
7072
188
11789
198
11868
161
11988
151
12003
222
12580
226
12636
31
12694
290
13331
225
42266
3
42646
315
77234
329
77325
111
78392
334
78809
115
79320
112
80399
1
80684
135
80900
7
119916
122
120016
124
120031
406
120246
382
120888
405
121954
408
122920
399
123464
376
124507
374
125306
297
125394
299
125409
479
125596
484
126853
205
126934
388
126949
501
127124
389
127311
209
127711
502
1080
Guanosine monophosphate
HMDB0001397
Guanosine monophosphate, also known as guanylic acid or 5'-GMP, belongs to the class of organic compounds known as purine ribonucleoside monophosphates. These are nucleotides consisting of a purine base linked to a ribose to which one monophosphate group is attached. Guanosine monophosphate is slightly soluble (in water) and a moderately acidic compound (based on its pKa). Guanosine monophosphate has been found throughout most human tissues, and has also been detected in multiple biofluids, such as saliva and blood. Guanosine monophosphate can be found anywhere throughout the human cell, such as in mitochondria, golgi, lysosome, and nucleus. Guanosine monophosphate exists in all living organisms, ranging from bacteria to humans. In humans, guanosine monophosphate is involved in the tigecycline action pathway, the homocarnosinosis pathway, the chloramphenicol action pathway, and the azithromycin action pathway. Guanosine monophosphate is also involved in several metabolic disorders, some of which include adenylosuccinate lyase deficiency, xanthine dehydrogenase deficiency (xanthinuria), adenine phosphoribosyltransferase deficiency (aprt), and myoadenylate deaminase deficiency. Outside of the human body, guanosine monophosphate can be found in a number of food items such as black salsify, oyster mushroom, endive, and redcurrant. This makes guanosine monophosphate a potential biomarker for the consumption of these food products. Guanosine 5\'-monophosphate. A guanine nucleotide containing one phosphate group esterified to the sugar moiety and found widely in nature.
85-32-5
C00144
6804
17345
GMP
6545
DB01972
NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1
C10H14N5O8P
InChI=1S/C10H14N5O8P/c11-10-13-7-4(8(18)14-10)12-2-15(7)9-6(17)5(16)3(23-9)1-22-24(19,20)21/h2-3,5-6,9,16-17H,1H2,(H2,19,20,21)(H3,11,13,14,18)/t3-,5-,6-,9-/m1/s1
RQFCJASXJCIDSX-UUOKFMHZSA-N
{[(2R,3S,4R,5R)-5-(2-amino-6-oxo-6,9-dihydro-1H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}phosphonic acid
363.2206
363.057998961
-2.01
6
guanylate
0
-2
FDB012390
5'-gmp;E 626;Gmp;Guanidine monophosphate;Guanosine 5'-monophosphate;Guanosine 5'-phosphate;Guanosine 5'-phosphorate;Guanosine 5'-phosphoric acid;Guanosine monophosphate;Guanosine-5'-monophosphate;Guanosine-5'-phosphate;Guanosine-phosphate;Guanylate;Guanylic acid;Pg;Guanosine 5'-monophosphoric acid;Guanosine monophosphoric acid;Guanosine-5'-monophosphoric acid
PW_C001080
GSMP
1732
8
1766
49
11770
198
11772
213
78005
111
78668
331
80685
135
120165
122
121849
383
124402
398
125525
297
127060
205
915
Nicotinic acid adenine dinucleotide
HMDB0001179
Nicotinic acid adenine dinucleotide phosphate, (NAADP), is a Ca2+-mobilizing second messenger synthesized in response to extracellular stimuli. NAADP binds to and opens Ca2+ channels on intracellular organelles, thereby increasing the intracellular Ca2+ concentration which, in turn, modulates a variety of cellular processes. Structurally, it is a dinucleotide that only differs from the house-keeping enzyme cofactor, NADP by a hydroxyl group (replacing the nicotinamide amino group) and yet this minor modification converts it into the most potent Ca2+-mobilizing second messenger yet described.
6450-77-7
C00857
165491
18304
DEAMIDO-NAD
145043
NC1=C2N=CN([C@@H]3O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]4O[C@H]([C@H](O)[C@@H]4O)[N+]4=CC=CC(=C4)C(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1
C21H27N6O15P2
InChI=1S/C21H26N6O15P2/c22-17-12-18(24-7-23-17)27(8-25-12)20-16(31)14(29)11(41-20)6-39-44(36,37)42-43(34,35)38-5-10-13(28)15(30)19(40-10)26-3-1-2-9(4-26)21(32)33/h1-4,7-8,10-11,13-16,19-20,28-31H,5-6H2,(H4-,22,23,24,32,33,34,35,36,37)/p+1/t10-,11-,13-,14-,15-,16-,19-,20-/m1/s1
SENPVEZBRZQVST-HISDBWNOSA-O
1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carboxy-1λ⁵-pyridin-1-ylium
665.4178
665.100962248
-2.57
8
deamido-NAD(+)
1
-2
FDB022469
Deamido-nad+;Deamidonicotinamide adenine dinucleoetide;Deamino-nad+;Naad;Naadp;Naadn;Nicotinate adenine dinucleotide;Nicotinic acid adenine dinucleotide;Nicotinic acid adenine dinucleotide phosphate;Deamido-nad
PW_C000915
NctAADn
2366
2
4871
8
5819
108
42665
315
78006
111
78394
132
80687
135
120167
122
121960
124
124513
118
125527
297
127062
205
127717
388
721
NAD
HMDB0000902
NAD (or Nicotinamide adenine dinucleotide) is used extensively in glycolysis and the citric acid cycle of cellular respiration. The reducing potential stored in NADH can be converted to ATP through the electron transport chain or used for anabolic metabolism. ATP "energy" is necessary for an organism to live. Green plants obtain ATP through photosynthesis, while other organisms obtain it by cellular respiration. (wikipedia). Nicotinamide adenine dinucleotide is a A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed).
53-84-9
C00003
5893
15846
NAD
5682
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
C21H28N7O14P2
InChI=1S/C21H27N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1-4,7-8,10-11,13-16,20-21,29-32H,5-6H2,(H5-,22,23,24,25,33,34,35,36,37)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1
BAWFJGJZGIEFAR-NNYOXOHSSA-O
1-[(2R,3R,4S,5R)-5-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
664.433
664.116946663
-2.59
8
1-[(2R,3R,4S,5R)-5-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
1
-1
FDB022309
3-carbamoyl-1-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;3-carbamoyl-1-beta-d-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-beta-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate inner salt;3-carbamoyl-1-delta-ribofuranosylpyridinium hydroxide 5'-ester with adenosine 5'-pyrophosphate;Adenine-nicotinamide dinucleotide;Co-i;Codehydrase i;Codehydrogenase i;Coenzyme i;Cozymase;Cozymase i;Diphosphopyridine nucleotide;Diphosphopyridine nucleotide oxidized;Endopride;Nad trihydrate;Nad-oxidized;Nicotinamide adenine dinucleotide;Nicotinamide adenine dinucleotide oxidized;Nicotinamide dinucleotide;Nicotineamide adenine dinucleotide;Oxidized diphosphopyridine nucleotide;Pyridine nucleotide diphosphate;[(3s,2r,4r,5r)-5-(6-aminopurin-9-yl)-3,4-dihydroxyoxolan-2-yl]methyl {[(3s,2r,4r,5r)-5-(3-carbamoylpyridyl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxyphosphoryl) hydrogen phosphate;[adenylate-32-p]-nad;Beta-diphosphopyridine nucleotide;Beta-nad;Beta-nicotinamide adenine dinucleotide;Beta-nicotinamide adenine dinucleotide trihydrate;Dpn;Nad;Nad+;Nadide;B-nad;β-nad
PW_C000721
NAD
140
4
150
3
353
8
651
10
1114
2
1134
43
1273
5
1466
54
2229
49
2779
17
2835
29
3107
9
4807
18
4813
18
4819
28
4902
6
4960
31
5167
95
5238
103
5334
111
5360
112
5469
123
5482
125
5590
135
5610
118
5696
100
5738
108
5827
141
5912
147
5942
151
6024
155
6072
157
6076
161
6385
1
6469
178
6772
117
6890
160
7012
188
7097
163
7174
205
7197
206
7405
198
7459
222
8241
226
8359
225
9085
224
11819
216
12322
249
13006
298
13018
300
13256
223
42404
322
42619
315
77104
132
77120
133
77209
134
77370
331
77650
336
77667
334
77702
332
77709
130
77915
113
77983
347
78406
356
80006
368
80690
119
93825
124
110552
388
112750
166
112853
94
119929
122
119952
406
120171
407
120834
419
120984
408
121159
425
121242
126
121259
429
121817
383
122614
384
122742
120
123130
447
123141
136
123419
455
123549
374
123731
460
123812
443
123829
464
124370
398
125187
121
125319
297
125342
479
125530
481
125806
299
125825
490
125924
482
126515
495
126765
480
126885
501
127278
507
127383
502
128089
390
128360
391
128428
395
1003
1-Pyrroline-5-carboxylic acid
HMDB0001301
1-Pyrroline-5-carboxylic acid is an enamine or an imino acid that forms upon the spontaneous dehydration of L-glutamate gamma-semialdehyde in aqueous solutions. The stereoisomer (S)-1-pyrroline-5-carboxylate is an intermediate in glutamate metabolism, arginine degradation, and proline biosynthesis and degradation. It can also be converted into or be formed from three amino acids: L-glutamate, L-ornithine, and L-proline. In particular, it is synthesized via the oxidation of proline by pyrroline-5-carboxylate reductase 1 (PYCR1) (EC 1.5.1.2) or by proline dehydrogenase (PRODH) (EC 1.5.99.8). It is hydrolyzed to L-glutamate by delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) (EC 1.5.1.12). It is also one of the few metabolites that can act as a precursor to other metabolites of both the urea cycle and the tricarboxylic acid (TCA) cycle. Under certain conditions, pyrroline-5-carboxylate can act as a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of pyrroline-5-carboxylate are associated with at least five inborn errors of metabolism, including hyperprolinemia type I, hyperprolinemia type II, iminoglycinuria, prolinemia type II, and pyruvate carboxylase deficiency. Hyperprolinemia type II results in high levels of pyrroline-5-carboxylate. People with hyperprolinemia type II have signs and symptoms that vary in severity, but they are more likely than type I to have seizures or intellectual disability. Pyrroline-5-carboxylate is highly reactive and excess quantities have been shown to cause cell death and apoptosis (PMID: 15548746).
2906-39-0
C04322
1196
1372
l-delta(1)-pyrroline_5-carboxylate
1159
OC(=O)C1CCC=N1
C5H7NO2
InChI=1S/C5H7NO2/c7-5(8)4-2-1-3-6-4/h3-4H,1-2H2,(H,7,8)
DWAKNKKXGALPNW-UHFFFAOYSA-N
3,4-dihydro-2H-pyrrole-2-carboxylic acid
113.1146
113.047678473
-0.94
1
1-pyrroline-5-carboxylic acid
0
-1
FDB022542
(s)-1-pyrroline-5-carboxylate;1-pyrroline-5-carboxylate;1-pyrroline-5-carboxylic acid;3,4-dihydro-2h-pyrrole-2-carboxylate;3,4-dihydro-2h-pyrrole-2-carboxylic acid;D1-pyrroline-5-carboxylate;D1-pyrroline-5-carboxylic acid;Dl-1-pyrroline-5-carboxylate;Dl-1-pyrroline-5-carboxylic acid;L-1-pyrroline-5-carboxylate;L-delta 1-pyrroline-5-carboxylate;Pyrroline 5-carboxylate;Pyrroline-5-carboxylate;Delta(1)pyrroline-5-carboxylate;Delta-1-pyrroline-5-carboxylate;Delta-1-pyrroline-5-carboxylic acid;Delta(1)-pyrroline-5-carboxylic acid;Delta(1)-pyrroline-5-carboxylate;δ(1)-pyrroline-5-carboxylate;δ(1)-pyrroline-5-carboxylic acid
PW_C001003
1P5CA
155
4
4873
8
4874
3
11859
161
77477
133
78007
112
80689
119
120170
407
120375
406
123020
120
125529
481
127064
206
1144
NADH
HMDB0001487
NADH is the reduced form of NAD+, and NAD+ is the oxidized form of NADH, A coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). It forms NADP with the addition of a phosphate group to the 2' position of the adenosyl nucleotide through an ester linkage.(Dorland, 27th ed).
58-68-4
C00004
439153
16908
NADH
388299
DB00157
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
C21H29N7O14P2
InChI=1S/C21H29N7O14P2/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(32)14(30)11(41-21)6-39-44(36,37)42-43(34,35)38-5-10-13(29)15(31)20(40-10)27-3-1-2-9(4-27)18(23)33/h1,3-4,7-8,10-11,13-16,20-21,29-32H,2,5-6H2,(H2,23,33)(H,34,35)(H,36,37)(H2,22,24,25)/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1
BOPGDPNILDQYTO-NNYOXOHSSA-N
[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy]({[(2R,3S,4R,5R)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy})phosphinic acid
665.441
665.124771695
-2.35
8
NADH
0
-2
FDB022649
1,4-dihydronicotinamide adenine dinucleotide;Dpnh;Dihydrocodehydrogenase i;Dihydrocozymase;Dihydronicotinamide adenine dinucleotide;Dihydronicotinamide mononucleotide;Enada;Nadh;Nadh2;Reduced codehydrogenase i;Reduced diphosphopyridine nucleotide;Reduced nicotinamide adenine diphosphate;Reduced nicotinamide-adenine dinucleotide;B-dpnh;B-nadh;Beta-dpnh;Beta-nadh;Nicotinamide adenine dinucleotide (reduced);Reduced nicotinamide adenine dinucleotide
PW_C001144
NADH
143
4
153
3
490
8
648
10
1115
2
1275
5
1469
54
2230
49
2781
17
2836
29
3109
9
4806
18
4812
18
4821
28
4904
6
4959
31
5169
95
5240
103
5332
111
5358
112
5466
123
5479
125
5593
135
5698
100
5737
108
5829
141
5915
147
5945
151
6027
155
6079
161
6387
1
6472
178
6771
117
6893
160
7011
188
7099
163
7172
205
7195
206
7462
222
8244
226
8360
225
9086
224
11809
198
11821
216
12320
249
13003
298
13015
300
13255
223
42403
322
42618
315
77107
132
77123
133
77208
134
77371
331
77651
336
77668
334
77700
332
77707
130
77917
113
77986
347
80009
368
80691
119
93822
124
110549
388
112854
94
115838
118
119955
406
120172
407
120378
122
120986
408
121162
425
121244
126
121693
429
121818
383
122616
384
122745
120
123127
447
123138
136
123551
374
123734
460
123814
443
124242
464
124371
398
125189
121
125345
479
125531
481
125762
297
125808
299
125926
482
126516
495
126767
480
126888
501
127385
502
128090
390
128362
391
128429
395
105
L-Alanine
HMDB0000161
Alanine is a non-essential amino acid made in the body from either the conversion of the carbohydrate pyruvate or the breakdown of DNA and the dipeptides carnosine and anserine. It is highly concentrated in muscle and is one of the most important amino acids released by muscle, functioning as a major energy source. Plasma alanine is often decreased when the BCAA (branched-chain amino acids) are deficient. This finding may relate to muscle metabolism. Alanine is highly concentrated in meat products and other high-protein foods like wheat germ and cottage cheese. Alanine is an important participant as well as a regulator of glucose metabolism. Alanine levels parallel blood sugar levels in both diabetes and hypoglycemia, and alanine reduces both severe hypoglycemia and the ketosis of diabetes. It is an important amino acid for lymphocyte reproduction and immunity. Alanine therapy has helped dissolve kidney stones in experimental animals. Normal alanine metabolism, like that of other amino acids, is highly dependent upon enzymes that contain vitamin B6. Alanine, like GABA, taurine, and glycine, is an inhibitory neurotransmitter in the brain (http://www.dcnutrition.com/AminoAcids/). L-Alanine has been found to be associated with glucagon deficiency, which is an inborn error of metabolism.
56-41-7
C00041
5950
16977
L-ALPHA-ALANINE
5735
DB00160
C[C@H](N)C(O)=O
C3H7NO2
InChI=1S/C3H7NO2/c1-2(4)3(5)6/h2H,4H2,1H3,(H,5,6)/t2-/m0/s1
QNAYBMKLOCPYGJ-REOHCLBHSA-N
(2S)-2-aminopropanoic acid
89.0932
89.047678473
0.70
2
L-alanine
0
0
FDB000556
(2s)-2-aminopropanoate;(2s)-2-aminopropanoic acid;(s)-(+)-alanine;(s)-2-aminopropanoate;(s)-2-aminopropanoic acid;(s)-2-amino-propanoate;(s)-2-amino-propanoic acid;(s)-alanine;2-aminopropanoate;2-aminopropanoic acid;2-aminopropionate;2-aminopropionic acid;2-ammoniopropanoate;2-ammoniopropanoic acid;Ala;Alanine;L-(+)-alanine;L-2-aminopropanoate;L-2-aminopropanoic acid;L-2-aminopropionate;L-2-aminopropionic acid;L-a-alanine;L-a-aminopropionate;L-a-aminopropionic acid;L-alpha-alanine;L-alpha-aminopropionate;L-alpha-aminopropionic acid;A-alanine;A-aminopropionate;A-aminopropionic acid;Alpha-alanine;Alpha-aminopropanoate;Alpha-aminopropanoic acid;Alpha-aminopropionate;Alpha-aminopropionic acid;A;L-alanin;L-α-alanine
PW_C000105
Ala
10
2
29
4
316
8
1446
50
1453
51
1454
26
3022
15
3439
3
5407
117
5418
103
5431
118
5452
120
5557
132
5578
133
5637
107
5638
108
5883
105
6529
85
8350
225
12271
151
12620
31
12627
18
15230
222
42452
320
42453
318
42534
315
77969
346
77975
327
77988
326
78008
111
78092
112
79165
114
80693
135
119910
122
120015
124
120026
406
121145
423
121151
424
121164
416
121220
409
122139
407
123717
458
123723
459
123736
452
123790
137
124691
119
125300
297
125393
299
125404
479
126296
481
126850
205
126933
388
126944
501
127860
206
134
Oxoglutaric acid
HMDB0000208
Oxoglutaric acid, also known as alpha-ketoglutarate, alpha-ketoglutaric acid, AKG, or 2-oxoglutaric acid, is classified as a gamma-keto acid or a gamma-keto acid derivative. gamma-Keto acids are organic compounds containing an aldehyde substituted with a keto group on the C4 carbon atom. alpha-Ketoglutarate is considered to be soluble (in water) and acidic. alpha-Ketoglutarate is a key molecule in the TCA cycle, playing a fundamental role in determining the overall rate of this important metabolic process (PMID: 26759695). In the TCA cycle, AKG is decarboxylated to succinyl-CoA and carbon dioxide by AKG dehydrogenase, which functions as a key control point of the TCA cycle. Additionally, AKG can be generated from isocitrate by oxidative decarboxylation catalyzed by the enzyme known as isocitrate dehydrogenase (IDH). In addition to these routes of production, AKG can be produced from glutamate by oxidative deamination via glutamate dehydrogenase, and as a product of pyridoxal phosphate-dependent transamination reactions (mediated by branched-chain amino acid transaminases) in which glutamate is a common amino donor. AKG is a nitrogen scavenger and a source of glutamate and glutamine that stimulates protein synthesis and inhibits protein degradation in muscles. In particular, AKG can decrease protein catabolism and increase protein synthesis to enhance bone tissue formation in skeletal muscles (PMID: 26759695). Interestingly, enteric feeding of AKG supplements can significantly increase circulating plasma levels of hormones such as insulin, growth hormone, and insulin-like growth factor-1 (PMID: 26759695). It has recently been shown that AKG can extend the lifespan of adult C. elegans by inhibiting ATP synthase and TOR (PMID: 24828042). In combination with molecular oxygen, alpha-ketoglutarate is required for the hydroxylation of proline to hydroxyproline in the production of type I collagen. A recent study has shown that alpha-ketoglutarate promotes TH1 differentiation along with the depletion of glutamine thereby favouring Treg (regulatory T-cell) differentiation (PMID: 26420908). alpha-Ketoglutarate has been found to be associated with fumarase deficiency, 2-ketoglutarate dehydrogenase complex deficiency, and D-2-hydroxyglutaric aciduria, which are all inborn errors of metabolism (PMID: 8338207).
328-50-7
C00026
51
30915
2-KETOGLUTARATE
50
DB02926
OC(=O)CCC(=O)C(O)=O
C5H6O5
InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)
KPGXRSRHYNQIFN-UHFFFAOYSA-N
2-oxopentanedioic acid
146.0981
146.021523302
-0.44
2
oxoglutarate
0
-2
FDB003361
2-ketoglutarate;2-ketoglutaric acid;2-oxo-1,5-pentanedioate;2-oxo-1,5-pentanedioic acid;2-oxoglutarate;2-oxoglutaric acid;2-oxopentanedioate;2-oxopentanedioic acid;Oxoglutarate;Alpha-ketoglutaric acid;Oxoglutaric acid;A-ketoglutarate;A-ketoglutaric acid;Alpha-ketoglutarate;α-ketoglutarate;α-ketoglutaric acid
PW_C000134
AKG
15
2
42
3
141
4
146
8
499
18
673
31
1108
42
1263
5
1447
50
1455
26
1467
54
5375
103
5414
117
5438
118
5564
132
6008
147
6036
155
6069
157
6092
161
6482
178
6530
85
7471
222
7515
224
7519
151
8209
225
8374
220
11863
198
12681
289
77054
253
77135
133
77481
111
77523
112
77746
129
77967
345
77970
346
77976
327
77984
347
78425
334
80018
368
80694
135
113162
94
119972
406
120022
124
120084
407
120174
122
120552
414
120814
418
120989
408
121146
423
121152
424
121160
425
122757
120
122831
119
123186
450
123399
454
123554
374
123718
458
123724
459
123732
460
125357
479
125400
299
125455
481
125533
297
125800
489
125929
482
126900
501
126940
388
126993
206
127066
205
127255
506
127388
502
164
Pyruvic acid
HMDB0000243
Pyruvic acid is an intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed.) Biological Source: Intermediate in primary metabolism including fermentation processes. Present in muscle in redox equilibrium with Lactic acid. A common constituent, as a chiral cyclic acetal linked to saccharide residues, of bacterial polysaccharides. Isolated from cane sugar fermentation broth and peppermint. Constituent of Bauhinia purpurea, Cicer arietinum (chickpea), Delonix regia, Pisum sativum (pea) and Trigonella caerulea (sweet trefoil) Use/Importance: Reagent for regeneration of carbonyl compdounds from semicarbazones, phenylhydrazones and oximes. Flavoring ingredient (Dictionary of Organic Compounds).
127-17-3
C00022
1060
32816
PYRUVATE
1031
DB00119
CC(=O)C(O)=O
C3H4O3
InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)
LCTONWCANYUPML-UHFFFAOYSA-N
2-oxopropanoic acid
88.0621
88.016043994
0.18
1
pyruvic acid
0
-1
FDB008293
2-oxopropanoate;2-oxopropanoic acid;2-oxopropionate;2-oxopropionic acid;Acetylformate;Acetylformic acid;Bts;Pyroracemate;Pyroracemic acid;Pyruvate;A-ketopropionate;A-ketopropionic acid;Alpha-ketopropionate;Alpha-ketopropionic acid;2-ketopropionic acid;2-oxopropansaeure;2-oxopropionsaeure;Acide pyruvique;Alpha-oxopropionsaeure;Brenztraubensaeure;Ch3cocooh;2-ketopropionate;α-ketopropionate;α-ketopropionic acid;A-oxopropionsaeure;α-oxopropionsaeure
PW_C000164
Pyr
17
2
20
4
422
8
1181
3
1449
50
1457
26
5365
103
5405
117
5440
118
5444
120
5566
132
5570
133
5893
95
5920
147
5951
151
6022
155
6067
156
6074
161
6126
160
6383
1
6467
178
6510
177
6532
85
7457
222
7495
220
8200
225
12622
31
15292
249
15349
18
77310
111
77972
346
77978
327
78090
112
80004
368
80042
367
80695
135
112879
94
115683
121
119950
406
120011
124
120175
122
120878
407
121148
423
121154
424
123454
119
123720
458
123726
459
125340
479
125390
299
125534
297
125854
481
126883
501
126931
388
127067
205
127858
206
1148
Pyridoxal 5'-phosphate
HMDB0001491
This is the active form of vitamin B6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (pyridoxamine). -- Pubchem; Pyridoxal-phosphate (PLP, pyridoxal-5'-phosphate) is a cofactor of many enzymatic reactions. It is the active form of vitamin B6 which comprises three natural organic compounds, pyridoxal, pyridoxamine and pyridoxine. -- Wikipedia.
54-47-7
C00018
1051
18405
PYRIDOXAL_PHOSPHATE
1022
DB00114
CC1=NC=C(COP(O)(O)=O)C(C=O)=C1O
C8H10NO6P
InChI=1S/C8H10NO6P/c1-5-8(11)7(3-10)6(2-9-5)4-15-16(12,13)14/h2-3,11H,4H2,1H3,(H2,12,13,14)
NGVDGCNFYWLIFO-UHFFFAOYSA-N
[(4-formyl-5-hydroxy-6-methylpyridin-3-yl)methoxy]phosphonic acid
247.1419
247.024573569
-1.64
3
pyridoxal phosphate
0
-2
FDB021820
Apolon b6;Biosechs;Codecarboxylase;Coenzyme b6;Hairoxal;Hexermin-p;Hi-pyridoxin;Hiadelon;Himitan;Pal-p;Plp;Phosphopyridoxal;Phosphopyridoxal coenzyme;Pidopidon;Piodel;Pydoxal;Pyridoxal 5'-phosphate;Pyridoxal 5-phosphate;Pyridoxal p;Pyridoxal phosphate;Pyridoxal-p;Pyridoxyl phosphate;Pyromijin;Sechvitan;Vitahexin-p;Vitazechs;3-hydroxy-2-methyl-5-[(phosphonooxy)methyl]-4-pyridinecarboxaldehyde;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphate;Phosphoric acid mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphoric acid ester;Pyridoxal 5'-(dihydrogen phosphate);Pyridoxal-5'-phosphate;Pyridoxal 5'-phosphoric acid;3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphoric acid;Phosphate mono-(4-formyl-5-hydroxy-6-methyl-pyridin-3-ylmethyl) ester;Pyridoxal 5-monophosphate ester;Pyridoxal 5'-(dihydrogen phosphoric acid);Pyridoxal 5-phosphoric acid;Pyridoxal phosphoric acid;Pyridoxal-5'-phosphoric acid
PW_C001148
Pyr-5'P
18
2
32
4
45
3
51
8
122
1
401
19
696
20
1110
42
1450
50
1458
26
2120
10
2150
49
5325
111
5416
117
5421
103
5441
118
5455
120
5567
132
5581
133
6533
85
7018
160
7167
205
7216
212
7222
213
11858
161
12175
151
12623
31
12628
18
12684
289
12689
290
77017
253
77037
225
77041
293
77052
224
77526
112
77764
341
77973
346
77979
327
78292
345
78855
332
78862
331
80696
135
98630
7
119912
122
120024
124
120029
406
120087
407
120817
418
121149
423
121155
424
122069
123
122076
383
122834
119
123402
454
123721
458
123727
459
124620
447
124627
398
125302
297
125402
299
125407
479
125458
481
125803
489
126224
298
126231
495
126942
388
126947
501
126996
206
127258
506
127786
513
127793
390
123
L-Aspartic acid
HMDB0000191
Aspartic acid (Asp, D), also known as aspartate, the name of its anion, is one of the 20 natural proteinogenic amino acids which are the building blocks of proteins. As its name indicates, aspartic acid is the carboxylic acid analog of asparagine. As a neurotransmitter, aspartic acid may provide resistance to fatigue and thus lead to endurance, although the evidence to support this idea is not strong (Wikipedia). Aspartic acid is a nonessential amino acid that is made from glutamic acid by enzymes using vitamin B6. The amino acid has important roles in the urea cycle and DNA metabolism. Aspartic acid is a major excitatory neurotransmitter, which is sometimes found to be increased in epileptic and stroke patients. It is decreased in depressed patients and in patients with brain atrophy. Aspartic acid supplements are being evaluated. Five grams can raise blood levels. Magnesium and zinc may be natural inhibitors of some of the actions of aspartic acid. Aspartic acid, with the amino acid phenylalanine, is a part of a new natural sweetener, aspartame. This sweetener is an advance in artificial sweeteners, and is probably safe in normal doses to all except phenylketonurics. The jury is still out on the long-term effects it has on many brain neurohormones. Aspartic acid may be a significant immunostimulant of the thymus and can protect against some of the damaging effects of radiation. Many claims have been made for the special value of administering aspartic acid in the form of potassium and magnesium salts. Since aspartic acid is relatively nontoxic, studies are now in progress to elucidate its pharmacological and therapeutic roles (http://www.dcnutrition.com/AminoAcids).
56-84-8
C00049
44367445
17053
L-ASPARTATE
5745
DB00128
N[C@@H](CC(O)=O)C(O)=O
C4H7NO4
InChI=1S/C4H7NO4/c5-2(4(8)9)1-3(6)7/h2H,1,5H2,(H,6,7)(H,8,9)/t2-/m0/s1
CKLJMWTZIZZHCS-REOHCLBHSA-N
(2S)-2-aminobutanedioic acid
133.1027
133.037507717
0.03
3
L-aspartic acid
0
-1
FDB012567
Asp;(+)-aspartate;(+)-aspartic acid;(2s)-aspartate;(2s)-aspartic acid;(l)-aspartate;(l)-aspartic acid;(r)-2-aminosuccinate;(s)-(+)-aspartate;(s)-(+)-aspartic acid;(s)-2-aminosuccinate;(s)-2-aminosuccinic acid;(s)-aminobutanedioate;(s)-aminobutanedioic acid;(s)-aspartate;(s)-aspartic acid;(s)-amino-butanedioate;(s)-amino-butanedioic acid;2-amino-3-methylsuccinate;2-amino-3-methylsuccinic acid;2-aminosuccinate;2-aminosuccinic acid;Aminosuccinate;Asparagate;Asparagic acid;Asparaginate;Asparaginic acid;Asparatate;Aspartate;H-asp-oh;L-(+)-aspartate;L-(+)-aspartic acid;L-aminosuccinate;L-aminosuccinic acid;L-asparagate;L-asparagic acid;L-asparaginate;L-asparaginic acid;L-aspartate;Alpha-aminosuccinate;Alpha-aminosuccinic acid;(s)-2-aminobutanedioic acid;Aspartic acid;D;L-asparaginsaeure;(s)-2-aminobutanedioate
PW_C000123
Asp
48
8
93
9
1101
3
1103
4
1104
42
1994
2
5663
107
5664
108
6851
188
7570
160
8185
225
11802
198
12686
290
12704
291
42363
318
42364
315
77323
111
77510
132
77532
113
78288
133
78289
345
79307
112
80699
135
119878
232
119881
117
120037
122
120421
124
120809
406
120813
418
123057
118
123394
120
123398
454
125415
297
125795
479
125799
489
126955
205
127250
501
127254
506
127555
388
148
Oxalacetic acid
HMDB0000223
Oxaloacetic acid, also known as oxosuccinic acid or oxalacetic acid, is a four-carbon dicarboxylic acid appearing as an intermediate of the citric acid cycle. In vivo, oxaloacetate (the ionized form of oxaloacetic acid) is formed by the oxidation of L-malate, catalyzed by malate dehydrogenase, and reacts with Acetyl-CoA to form citrate, catalyzed by citrate synthase.(wikipedia) A class of ketodicarboxylic acids derived from oxalic acid. Oxaloacetic acid is an intermediate in the citric acid cycle and is converted to aspartic acidD by a transamination reaction.
328-42-7
C00036
970
30744
OXALACETIC_ACID
945
OC(=O)CC(=O)C(O)=O
C4H4O5
InChI=1S/C4H4O5/c5-2(4(8)9)1-3(6)7/h1H2,(H,6,7)(H,8,9)
KHPXUQMNIQBQEV-UHFFFAOYSA-N
2-oxobutanedioic acid
132.0716
132.005873238
-0.36
2
oxalacetate
0
-2
FDB001479
2-ketosuccinate;2-ketosuccinic acid;2-oxobutanedioate;2-oxobutanedioic acid;2-oxosuccinate;2-oxosuccinic acid;Ketosuccinate;Ketosuccinic acid;Oaa;Oxalacetate;Oxaloacetate;Oxaloacetic acid;Oxaloethanoate;Oxaloethanoic acid;Oxosuccinate;Oxosuccinic acid;A-ketosuccinate;A-ketosuccinic acid;Alpha-ketosuccinate;Alpha-ketosuccinic acid;3-carboxy-3-oxopropanoic acid;Keto-succinic acid;Oxalacetic acid;Oxobutanedioic acid;3-carboxy-3-oxopropanoate;Keto-succinate;Oxobutanedioate
PW_C000148
Oaa
25
4
96
9
111
5
1099
3
1109
42
1113
2
1688
8
5371
103
5448
120
5574
133
6033
155
6088
161
6478
178
7468
222
7513
224
7517
151
8372
220
8378
225
11744
117
11891
160
12707
291
12717
292
43792
322
77508
132
77533
113
77538
334
77958
112
78009
111
78290
345
80015
368
80700
135
119964
406
120048
408
120062
126
120180
122
120419
124
120815
418
121207
407
122799
374
122813
443
123055
118
123400
454
123777
119
125354
479
125426
482
125439
301
125537
297
125801
489
125807
299
126897
501
126965
502
126977
207
127070
205
127256
506
127261
388
448
L-Cysteine
HMDB0000574
Cysteine is a naturally occurring, sulfur-containing amino acid that is found in most proteins, although only in small quantities. Cysteine is unique amongst the twenty natural amino acids as it contains a thiol group. Thiol groups can undergo oxidation/reduction (redox) reactions; when cysteine is oxidized it can form cystine, which is two cysteine residues joined by a disulfide bond. This reaction is reversible since the reduction of this disulphide bond regenerates two cysteine molecules. The disulphide bonds of cystine are crucial to defining the structures of many proteins. Cysteine is often involved in electron-transfer reactions, and help the enzyme catalyze its reaction. Cysteine is also part of the antioxidant glutathione. N-Acetyl-L-cysteine (NAC) is a form of cysteine where an acetyl group is attached to cysteine's nitrogen atom and is sold as a dietary supplement. Cysteine is named after cystine, which comes from the Greek word kustis meaning bladder (cystine was first isolated from kidney stones). Oxidation of cysteine can produce a disulfide bond with another thiol and further oxidation can produce sulphfinic or sulfonic acids. The cysteine thiol group is also a nucleophile and can undergo addition and substitution reactions. Thiol groups become much more reactive when they are ionized, and cysteine residues in proteins have pKa values close to neutrality, so they are often in their reactive thiolate form in the cell. The thiol group also has a high affinity for heavy metals and proteins containing cysteine will bind metals such as mercury, lead, and cadmium tightly. Due to this ability to undergo redox reactions, cysteine has antioxidant properties. Cysteine is an important source of sulfur in human metabolism, and although it is classified as a non-essential amino acid, cysteine may be essential for infants, the elderly, and individuals with certain metabolic disease or who suffer from malabsorption syndromes. Cysteine may at some point be recognized as an essential or conditionally essential amino acid (Wikipedia). Cysteine is important in energy metabolism. As cystine, it is a structural component of many tissues and hormones. Cysteine has clinical uses ranging from baldness to psoriasis to preventing smoker's hack. In some cases, oral cysteine therapy has proved excellent for treatment of asthmatics, enabling them to stop theophylline and other medications. Cysteine also enhances the effect of topically applied silver, tin, and zinc salts in preventing dental cavities. In the future, cysteine may play a role in the treatment of cobalt toxicity, diabetes, psychosis, cancer, and seizures (http://www.dcnutrition.com/AminoAcids/).
52-90-4
C00097
5862
17561
CYS
5653
DB00151
N[C@@H](CS)C(O)=O
C3H7NO2S
InChI=1S/C3H7NO2S/c4-2(1-7)3(5)6/h2,7H,1,4H2,(H,5,6)/t2-/m0/s1
XUJNEKJLAYXESH-REOHCLBHSA-N
(2R)-2-amino-3-sulfanylpropanoic acid
121.158
121.019749163
-0.72
3
L-cysteine
0
0
DBMET00503
FDB012678
(+)-2-amino-3-mercaptopropionic acid;(2r)-2-amino-3-mercaptopropanoate;(2r)-2-amino-3-mercaptopropanoic acid;(2r)-2-amino-3-sulfanylpropanoate;(2r)-2-amino-3-sulfanylpropanoic acid;(r)-(+)-cysteine;(r)-2-amino-3-mercaptopropanoate;(r)-2-amino-3-mercaptopropanoic acid;(r)-2-amino-3-mercapto-propanoate;(r)-2-amino-3-mercapto-propanoic acid;(r)-cysteine;2-amino-3-mercaptopropanoate;2-amino-3-mercaptopropanoic acid;2-amino-3-mercaptopropionate;2-amino-3-mercaptopropionic acid;3-mercapto-l-alanine;Acetylcysteine;B-mercaptoalanine;Carbocysteine;Cisteina;Cisteinum;Cystein;Cysteine;Cysteinum;Free cysteine;Half-cystine;L cysteine;L-(+)-cysteine;L-2-amino-3-mercaptopropanoate;L-2-amino-3-mercaptopropanoic acid;L-2-amino-3-mercaptopropionic acid;L-cystein;L-cysteine;Polycysteine;Thioserine;Alpha-amino-beta-thiolpropionic acid;Beta-mercaptoalanine;C;Cys;E920;L-zystein;(2r)-2-amino-3-sulphanylpropanoate;(2r)-2-amino-3-sulphanylpropanoic acid;L-2-amino-3-mercaptopropionate
PW_C000448
Cys
174
8
1867
2
2864
9
2870
15
5767
101
5801
108
6756
117
6759
107
7078
188
7496
224
7594
160
8256
227
8260
225
12012
281
12269
151
42651
315
43730
322
77778
111
77795
113
77796
132
80704
135
120125
122
120131
124
120580
126
122863
118
123210
443
125491
297
125498
299
127029
205
127035
388
826
γ-Glutamylcysteine
HMDB0001049
gamma-Glutamylcysteine is a dipeptide composed of gamma-glutamate and cysteine, and is a proteolytic breakdown product of larger proteins. It belongs to the family of N-acyl-alpha Amino Acids and Derivatives. These are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom. gamma-Glutamylcysteine is an incomplete breakdown product of protein digestion or protein catabolism. Some dipeptides are known to have physiological or cell-signaling effects although most are simply short-lived intermediates on their way to specific amino acid degradation pathways following further proteolysis. gamma-Glutamylcysteine is a product of enzyme glutamate-cysteine ligase [EC 6.3.2.2] and a substrate of enzyme glutathione synthase [EC 6.3.2.3] in the glutamate metabolism pathway (KEGG).
636-58-8
C00669
123938
17515
L-GAMMA-GLUTAMYLCYSTEINE
110467
DB03408
N[C@@H](CCC(=O)N[C@@H](CS)C(O)=O)C(O)=O
C8H14N2O5S
InChI=1S/C8H14N2O5S/c9-4(7(12)13)1-2-6(11)10-5(3-16)8(14)15/h4-5,16H,1-3,9H2,(H,10,11)(H,12,13)(H,14,15)/t4-,5-/m0/s1
RITKHVBHSGLULN-WHFBIAKZSA-N
(2S)-2-amino-4-{[(1R)-1-carboxy-2-sulfanylethyl]carbamoyl}butanoic acid
250.272
250.062342258
-1.98
5
gamma-glutamylcysteine
0
-1
FDB003603
(des-gly)-Glutathione;3gc;5-L-Glutamyl-L-cysteine;5-L-Glutamylcysteine;H-Glu(Cys-OH)-OH;H-gamma-Glu-Cys-OH;L-g-Glutamyl-L-cysteine;L-gamma-Glutamyl-L-cysteine;L-gamma-Glutamylcysteine;N-(1-Carboxy-2-mercaptoethyl)-L-glutamine;N-L-gamma-Glutamyl-L-cysteine;Xn-L-g-glutamyl-glutamine;Xn-L-gamma-glutamyl-glutamine;g-Glutamylcysteine;g-L-Glutamyl-L-cysteine;gamma-Glu-Cys;gamma-Glutamylcysteine;gamma-L-Glutamyl-L-cysteine;Gammaglucys;Glu(-Cys);L-γ-Glutamyl-L-cysteine;G-Glu-Cys;γ-Glu-Cys;γ-Glutamylcysteine;γ-L-Glutamyl-L-cysteine;L-g-Glutamylcysteine;L-γ-Glutamylcysteine;γ-L-Glu-L-Cys;N-γ-Glutamylcysteine;N-L-γ-Glutamyl-cysteine;N-L-γ-Glutamyl-L-cysteine;gamma-L-Glu-L-Cys;N-L-gamma-Glutamyl-cysteine;N-γ-L-Glutamyl-L-cysteine
PW_C000826
γGluCys
175
8
4662
2
77780
111
80705
135
120096
122
125465
297
127002
205
78
Glycine
HMDB0000123
Glycine is a simple, nonessential amino acid, although experimental animals show reduced growth on low-glycine diets. The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the body's production of DNA, phospholipids and collagen, and in release of energy. Glycine levels are effectively measured in plasma in both normal patients and those with inborn errors of glycine metabolism. (http://www.dcnutrition.com/AminoAcids/) Nonketotic hyperglycinaemia (OMIM 606899) is an autosomal recessive condition caused by deficient enzyme activity of the glycine cleavage enzyme system (EC 2.1.1.10). The glycine cleavage enzyme system comprises four proteins: P-, T-, H- and L-proteins (EC 1.4.4.2, EC 2.1.2.10 and EC 1.8.1.4 for P-, T- and L-proteins). Mutations have been described in the GLDC (OMIM 238300), AMT (OMIM 238310), and GCSH (OMIM 238330) genes encoding the P-, T-, and H-proteins respectively. The glycine cleavage system catalyses the oxidative conversion of glycine into carbon dioxide and ammonia, with the remaining one-carbon unit transferred to folate as methylenetetrahydrofolate. It is the main catabolic pathway for glycine and it also contributes to one-carbon metabolism. Patients with a deficiency of this enzyme system have increased glycine in plasma, urine and cerebrospinal fluid (CSF) with an increased CSF: plasma glycine ratio. (PMID 16151895).
56-40-6
C00037
5257127
15428
GLY
730
DB00145
NCC(O)=O
C2H5NO2
InChI=1S/C2H5NO2/c3-1-2(4)5/h1,3H2,(H,4,5)
DHMQDGOQFOQNFH-UHFFFAOYSA-N
2-aminoacetic acid
75.0666
75.032028409
0.87
2
glycine
0
0
FDB000484
2-aminoacetate;2-aminoacetic acid;Aciport;Amino-acetate;Amino-acetic acid;Aminoacetate;Aminoacetic acid;Aminoethanoate;Aminoethanoic acid;Glicoamin;Glycocoll;Glycolixir;Glycosthene;Gyn-hydralin;Padil;Aminoessigsaeure;G;Gly;Glycin;Glykokoll;Glyzin;H2n-ch2-cooh;Hgly;Leimzucker
PW_C000078
Gly
31
4
179
8
1812
2
1881
27
2829
29
5420
103
5454
120
5580
133
5640
107
5641
108
5863
105
6007
147
7014
160
7439
3
7441
166
7442
15
11794
198
11872
161
12429
151
15233
222
42419
318
42420
315
77644
336
77742
111
78022
132
78304
351
80708
135
120028
406
120097
122
120117
124
121687
429
122283
435
122850
118
124236
464
124837
470
125406
479
125466
297
125484
299
126448
499
126946
501
127003
205
127021
388
128018
517
80
Glutathione
HMDB0000125
Glutathione is a compound synthesized from cysteine, perhaps the most important member of the body's toxic waste disposal team. Like cysteine, glutathione contains the crucial thiol (-SH) group that makes it an effective antioxidant. There are virtually no living organisms on this planet-animal or plant whose cells don't contain some glutathione. Scientists have speculated that glutathione was essential to the very development of life on earth. glutathione has many roles; in none does it act alone. It is a coenzyme in various enzymatic reactions. The most important of these are redox reactions, in which the thiol grouping on the cysteine portion of cell membranes protects against peroxidation; and conjugation reactions, in which glutathione (especially in the liver) binds with toxic chemicals in order to detoxify them. glutathione is also important in red and white blood cell formation and throughout the immune system. glutathione's clinical uses include the prevention of oxygen toxicity in hyperbaric oxygen therapy, treatment of lead and other heavy metal poisoning, lowering of the toxicity of chemotherapy and radiation in cancer treatments, and reversal of cataracts. (http://www.dcnutrition.com/AminoAcids/) glutathione participates in leukotriene synthesis and is a cofactor for the enzyme glutathione peroxidase. It is also important as a hydrophilic molecule that is added to lipophilic toxins and waste in the liver during biotransformation before they can become part of the bile. glutathione is also needed for the detoxification of methylglyoxal, a toxin produced as a by-product of metabolism. This detoxification reaction is carried out by the glyoxalase system. Glyoxalase I (EC 4.4.1.5) catalyzes the conversion of methylglyoxal and reduced glutathione to S-D-Lactoyl-glutathione. Glyoxalase II (EC 3.1.2.6) catalyzes the hydrolysis of S-D-Lactoyl-glutathione to glutathione and D-lactate. GSH is known as a substrate in both conjugation reactions and reduction reactions, catalyzed by glutathione S-transferase enzymes in cytosol, microsomes, and mitochondria. However, it is also capable of participating in non-enzymatic conjugation with some chemicals, as in the case of n-acetyl-p-benzoquinone imine (NAPQI), the reactive cytochrome P450-reactive metabolite formed by acetaminophen, that becomes toxic when GSH is depleted by an overdose (of acetaminophen). glutathione in this capacity binds to NAPQI as a suicide substrate and in the process detoxifies it, taking the place of cellular protein thiol groups which would otherwise be covalently modified; when all GSH has been spent, NAPQI begins to react with the cellular proteins, killing the cells in the process. The preferred treatment for an overdose of this painkiller is the administration (usually in atomized form) of N-acetylcysteine, which is used by cells to replace spent GSSG and renew the usable GSH pool. (http://en.wikipedia.org/wiki/glutathione).
70-18-8
C00051
124886
16856
OXIDIZED-GLUTATHIONE
111188
DB00143
N[C@@H](CCC(=O)N[C@@H](CS)C(=O)NCC(O)=O)C(O)=O
C10H17N3O6S
InChI=1S/C10H17N3O6S/c11-5(10(18)19)1-2-7(14)13-6(4-20)9(17)12-3-8(15)16/h5-6,20H,1-4,11H2,(H,12,17)(H,13,14)(H,15,16)(H,18,19)/t5-,6-/m0/s1
RWSXRVCMGQZWBV-WDSKDSINSA-N
(2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-sulfanylethyl]carbamoyl}butanoic acid
307.323
307.083805981
-2.54
6
glutathione
0
-1
DBMET00487
FDB001498
5-l-glutamyl-l-cysteinylglycine;Agifutol s;Bakezyme rx;Copren;Deltathione;Gsh;Glutathion;Glutathione;Glutathione red;Glutathione reduced;Glutathione-sh;Glutatiol;Glutatione;Glutide;Glutinal;Isethion;L-glutamyl-l-cysteinylglycine;L-glutathione;L-glutathione reduce;L-g-glutamyl-l-cysteinyl-glycine;L-gamma-glutamyl-l-cysteinyl-glycine;L-gamma-glutamyl-l-cysteinylglycine;Ledac;Neuthion;Red. glutathione;Reduced glutathione;Tathion;Tathione;Triptide;Gamma-glutamylcysteinylglycine;Gamma-l-glutamyl-l-cysteinyl-glycine;Gamma-l-glutamyl-l-cysteinylglycine;N-(n-gamma-l-glutamyl-l-cysteinyl)glycine;G-l-glutamyl-l-cysteinyl-glycine;γ-l-glutamyl-l-cysteinyl-glycine;N-(n-g-l-glutamyl-l-cysteinyl)glycine;N-(n-γ-l-glutamyl-l-cysteinyl)glycine
PW_C000080
GSH
161
14
199
8
614
2
1337
10
1362
49
4889
31
5193
97
5839
143
8426
151
77403
332
77408
111
77424
331
78015
115
78761
132
80709
135
120098
405
120187
122
120880
124
121067
123
121093
383
122838
376
123456
118
123632
447
123658
398
125467
478
125544
297
125856
299
127004
209
127077
205
1847
Oxidized glutathione
HMDB0003337
Oxidized glutathione is a glutathione dimer formed by a disulfide bond between the cysteine sulfhydryl side chains during the course of being oxidized. glutathione participates in leukotriene synthesis and is a cofactor for the enzyme glutathione peroxidase. It is also important as a hydrophilic molecule that is added to lipophilic toxins and waste in the liver during biotransformation before they can become part of the bile. glutathione is also needed for the detoxification of methylglyoxal, a toxin produced as a by-product of metabolism. This detoxification reaction is carried out by the glyoxalase system. Glyoxalase I (EC 4.4.1.5) catalyzes the conversion of methylglyoxal and reduced glutathione to S-D-Lactoyl-glutathione. Glyoxalase II (EC 3.1.2.6) catalyzes the hydrolysis of S-D-Lactoyl-glutathione to glutathione and D-lactate.
27025-41-8
C00127
5255796
17858
OXIDIZED-GLUTATHIONE
950
NC(CCC(=O)NC(CSSCC(NC(=O)CCC(N)C(O)=O)C(=O)NCC(O)=O)C(=O)NCC(O)=O)C(O)=O
C20H32N6O12S2
InChI=1S/C20H32N6O12S2/c21-9(19(35)36)1-3-13(27)25-11(17(33)23-5-15(29)30)7-39-40-8-12(18(34)24-6-16(31)32)26-14(28)4-2-10(22)20(37)38/h9-12H,1-8,21-22H2,(H,23,33)(H,24,34)(H,25,27)(H,26,28)(H,29,30)(H,31,32)(H,35,36)(H,37,38)
YPZRWBKMTBYPTK-UHFFFAOYSA-N
2-amino-4-[(2-{[2-(4-amino-4-carboxybutanamido)-2-[(carboxymethyl)carbamoyl]ethyl]disulfanyl}-1-[(carboxymethyl)carbamoyl]ethyl)carbamoyl]butanoic acid
612.631
612.151961898
-3.18
10
oxiglutatione
0
-2
FDB023147
Gssg;Glutathione disulfide;L(-)-glutathione(oxidized);Oxiglutatione;(2s)-2-azaniumyl-4-{[(1r)-2-{[(2r)-2-[(4s)-4-azaniumyl-4-carboxylatobutanamido]-2-[(carboxylatomethyl)carbamoyl]ethyl]disulfanyl}-1-[(carboxylatomethyl)carbamoyl]ethyl]carbamoyl}butanoate
PW_C001847
GSSG
184
8
4878
3
77410
111
78012
112
80711
119
120103
122
120188
407
122840
135
125470
297
125545
481
127007
205
127078
206
146
NADPH
HMDB0000221
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.).
53-57-6
C00005
22833512
16474
NADPH
17215925
NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O
C21H30N7O17P3
InChI=1S/C21H30N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1,3-4,7-8,10-11,13-16,20-21,29-31H,2,5-6H2,(H2,23,32)(H,36,37)(H,38,39)(H2,22,24,25)(H2,33,34,35)/t10-,11-,13-,14-,15-,16-,20-,21-/m0/s1
ACFIXJIJDZMPPO-NCHANQSKSA-N
{[(2S,3S,4S,5S)-2-(6-amino-9H-purin-9-yl)-5-[({[({[(2S,3R,4S,5S)-5-(3-carbamoyl-1,4-dihydropyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-4-hydroxyoxolan-3-yl]oxy}phosphonic acid
745.4209
745.091102105
-2.14
9
[(2S,3S,4S,5S)-2-(6-aminopurin-9-yl)-5-{[({[(2S,3R,4S,5S)-5-(3-carbamoyl-4H-pyridin-1-yl)-3,4-dihydroxyoxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-4-hydroxyoxolan-3-yl]oxyphosphonic acid
0
-4
FDB021909
2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-b-d-ribofuranosylnicotinamide;2'-(dihydrogen phosphate) 5'-(trihydrogen pyrophosphate) adenosine 5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosylnicotinamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-d-ribofuranosyl-3-pyridinecarboxamide;Adenosine 5'-(trihydrogen diphosphate) 2'-(dihydrogen phosphate) p'-5'-ester with 1,4-dihydro-1-beta-delta-ribofuranosyl-3-pyridinecarboxamide;Dihydrocodehydrogenase ii;Dihydronicotinamide adenine dinucleotide phosphate;Dihydronicotinamide adenine dinucleotide-p;Dihydrotriphosphopyridine nucleotide reduced;Nadp-reduced;Nadph;Nicotinamide-adenine-dinucleotide-phosphorate;Nicotinamide-adenine-dinucleotide-phosphoric acid;Reduced codehydrase ii;Reduced coenzyme ii;Reduced cozymase ii;Reduced triphosphopyridine nucleotide;Triphosphopyridine nucleotide reduced;B-nadph;B-nicotinamide-adenine-dinucleotide-phosphorate;B-nicotinamide-adenine-dinucleotide-phosphoric acid;Beta-nadph;Beta-nicotinamide-adenine-dinucleotide-phosphorate;Beta-nicotinamide-adenine-dinucleotide-phosphoric acid;Nicotinamide adenine dinucleotide phosphate - reduced
PW_C000146
NADPH
185
8
190
3
778
10
796
5
821
18
837
2
1609
29
1615
49
4687
31
4793
14
4797
14
5310
111
5789
108
5972
147
6128
159
6271
35
6779
117
7068
188
7103
163
7154
205
7205
160
7315
213
7345
210
7559
212
7591
170
8194
225
8219
151
8421
224
11812
198
11893
211
12006
222
12150
164
12245
286
12596
226
12648
249
42343
315
43746
322
76911
293
77166
132
77385
331
77394
332
77460
130
77504
112
77511
115
77623
336
80712
119
113164
94
120105
407
120425
405
120452
122
120616
123
121141
125
121275
429
121402
124
121483
383
123059
376
123086
135
123241
447
123712
136
123846
464
123961
118
124041
398
125472
481
125696
297
126214
299
126529
495
127009
206
127572
388
128101
390
143
NADP
HMDB0000217
Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5-phosphate (NMN) coupled by pyrophosphate linkage to the 5-phosphate adenosine 2,5-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed.) Hydrogen carrier in biochemical redox systems. In the hexose monophosphoric acid system it is reduced to Dihydrocoenzyme II and reoxidation in the presence of flavoproteins (Dictionary of Organic Compounds).
53-59-8
C00006
5886
18009
NAD(P)
5675
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
C21H29N7O17P3
InChI=1S/C21H28N7O17P3/c22-17-12-19(25-7-24-17)28(8-26-12)21-16(44-46(33,34)35)14(30)11(43-21)6-41-48(38,39)45-47(36,37)40-5-10-13(29)15(31)20(42-10)27-3-1-2-9(4-27)18(23)32/h1-4,7-8,10-11,13-16,20-21,29-31H,5-6H2,(H7-,22,23,24,25,32,33,34,35,36,37,38,39)/p+1/t10-,11-,13-,14-,15-,16-,20-,21-/m1/s1
XJLXINKUBYWONI-NNYOXOHSSA-O
1-[(2R,3R,4S,5R)-5-[({[({[(2R,3R,4R,5R)-5-(6-amino-9H-purin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
744.4129
744.083277073
-2.27
9
1-[(2R,3R,4S,5R)-5-{[({[(2R,3R,4R,5R)-5-(6-aminopurin-9-yl)-3-hydroxy-4-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-3,4-dihydroxyoxolan-2-yl]-3-carbamoyl-1lambda5-pyridin-1-ylium
1
-3
FDB021908
Adenine-nicotinamide dinucleotide phosphate;Codehydrase ii;Codehydrogenase ii;Coenzyme ii;Cozymase ii;Nad phosphate;Nadp;Nadp+;Nicotinamide adenine dinucleotide phosphate;Nicotinamide-adenine dinucleotide phosphate;Tpn;Triphosphopyridine nucleotide;B-nadp;B-nicotinamide adenine dinucleotide phosphate;B-tpn;Beta-nadp;Beta-nicotinamide adenine dinucleotide phosphate;Beta-tpn;Oxidized nicotinamide-adenine dinucleotide phosphate;B-nicotinamide adenine dinucleotide phosphoric acid;Beta-nicotinamide adenine dinucleotide phosphoric acid;β-nicotinamide adenine dinucleotide phosphate;β-nicotinamide adenine dinucleotide phosphoric acid
PW_C000143
NADP
183
8
191
3
768
5
780
10
824
18
839
2
1611
29
1617
49
4685
31
4796
14
4801
14
5308
111
5790
108
6017
147
6132
159
6273
35
6778
117
7069
188
7105
163
7152
205
7206
160
7317
213
7346
210
7562
212
7589
170
8197
225
8220
151
8419
224
11811
198
11897
211
12008
222
12152
164
12249
286
12597
226
12650
249
42344
315
43745
322
76913
293
77164
132
77384
331
77396
332
77461
130
77515
115
77624
336
77814
334
77870
112
80713
119
113165
94
120106
407
120429
405
120450
122
120604
408
120618
123
121142
125
121277
429
121401
124
121485
383
123063
376
123084
135
123229
374
123243
447
123713
136
123848
464
123960
118
124043
398
125473
481
125694
297
125743
482
126215
299
126528
495
127010
206
127225
502
127570
388
128100
390
964
FAD
HMDB0001248
FAD, also known as flavitan or adeflavin, belongs to the class of organic compounds known as flavin nucleotides. These are nucleotides containing a flavin moiety. Flavin is a compound that contains the tricyclic isoalloxazine ring system, which bears 2 oxo groups at the 2- and 4-positions. FAD is a drug which is used to treat eye diseases caused by vitamin b2 deficiency, such as keratitis and blepharitis. FAD is slightly soluble (in water) and a moderately acidic compound (based on its pKa). FAD has been found in human liver and muscle tissues, and has also been detected in multiple biofluids, such as feces and blood. Within the cell, FAD is primarily located in the cytoplasm, mitochondria, endoplasmic reticulum and peroxisome. FAD exists in all living organisms, ranging from bacteria to humans. In humans, FAD is involved in the risedronate action pathway, the ibandronate action pathway, the valine, leucine and isoleucine degradation pathway, and the pyrimidine metabolism pathway. FAD is also involved in several metabolic disorders, some of which include the oncogenic action OF L-2-hydroxyglutarate in hydroxygluaricaciduria pathway, gaba-transaminase deficiency, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, and the saccharopinuria/hyperlysinemia II pathway. FAD is a condensation product of riboflavin and adenosine diphosphate. The coenzyme of various aerobic dehydrogenases, e.g., D-amino acid oxidase and L-amino acid oxidase. (Lehninger, Principles of Biochemistry, 1982, p972).
146-14-5
C00016
643975
16238
FAD
559059
DB03147
CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2
C27H33N9O15P2
InChI=1S/C27H33N9O15P2/c1-10-3-12-13(4-11(10)2)35(24-18(32-12)25(42)34-27(43)33-24)5-14(37)19(39)15(38)6-48-52(44,45)51-53(46,47)49-7-16-20(40)21(41)26(50-16)36-9-31-17-22(28)29-8-30-23(17)36/h3-4,8-9,14-16,19-21,26,37-41H,5-7H2,1-2H3,(H,44,45)(H,46,47)(H2,28,29,30)(H,34,42,43)/t14-,15+,16+,19-,20+,21+,26+/m0/s1
VWWQXMAJTJZDQX-UYBVJOGSSA-N
{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-3,4-dihydroxyoxolan-2-yl]methoxy}[({[(2R,3S,4S)-5-{7,8-dimethyl-2,4-dioxo-2H,3H,4H,10H-benzo[g]pteridin-10-yl}-2,3,4-trihydroxypentyl]oxy}(hydroxy)phosphoryl)oxy]phosphinic acid
785.5497
785.157134455
-2.27
9
flavine-adenine dinucleotide
0
-3
FDB022511
1h-purin-6-amine flavin dinucleotide;1h-purin-6-amine flavine dinucleotide;Adenine-flavin dinucleotide;Adenine-flavine dinucleotide;Adenine-riboflavin dinuceotide;Adenine-riboflavin dinucleotide;Adenine-riboflavine dinucleotide;Fad;Flamitajin b;Flanin f;Flavin adenine dinucleotide;Flavin adenine dinucleotide oxidized;Flavin-adenine dinucleotide;Flavine adenosine diphosphate;Flavine-adenine dinucleotide;Flavitan;Flaziren;Isoalloxazine-adenine dinucleotide;Riboflavin 5'-adenosine diphosphate;Riboflavin-adenine dinucleotide;Riboflavine-adenine dinucleotide;Adeflavin
PW_C000964
FAD
99
9
114
5
186
8
192
3
216
4
253
17
628
28
825
18
840
2
1188
14
1489
42
1612
29
1622
49
2133
58
2536
22
3723
26
4602
36
4688
31
4741
13
4758
10
4881
6
5268
103
5285
102
5335
111
5496
126
5511
127
5613
118
6030
155
6054
156
6082
161
6116
162
6390
1
6475
178
6499
179
6666
107
7039
163
7175
205
7321
213
7465
222
7487
223
9076
224
11818
216
11887
215
11899
211
12296
225
12328
249
12443
151
12519
227
12595
226
12710
291
12720
292
13029
301
13041
302
43623
318
77080
293
77126
133
77152
134
77501
113
77507
112
77518
115
77541
334
77615
132
77726
337
78054
329
78375
345
78930
331
79222
336
79272
358
80012
368
80034
369
80714
119
119958
406
119999
384
120051
408
120107
407
120432
405
120453
122
120490
124
121278
429
121298
418
121417
382
121489
383
122748
120
122776
121
122802
374
122823
443
123066
376
123087
135
123166
448
123849
464
123868
454
123976
399
124047
398
125348
479
125378
480
125429
482
125474
481
125697
297
125979
489
126107
299
126277
484
126891
501
126920
391
126968
502
126987
207
127011
206
127310
209
127432
506
127602
388
127840
389
71
γ-Aminobutyric acid
HMDB0000112
gamma-Aminobutyric acid (GABA) is an inhibitory neurotransmitter found in the nervous systems of widely divergent species, including humans. It is the chief inhibitory neurotransmitter in the vertebrate central nervous system. In vertebrates, GABA acts at inhibitory synapses in the brain. It acts by binding to specific transmembrane receptors in the plasma membrane of both pre- and postsynaptic neurons. This binding causes the opening of ion channels to allow either the flow of negatively-charged chloride ions into the cell or positively-charged potassium ions out of the cell. This will typically result in a negative change in the transmembrane potential, usually causing hyperpolarization. Three general classes of GABA receptor are known (PMID: 10561820). These include GABA-A and GABA-C ionotropic receptors, which are ion channels themselves, and GABA-B metabotropic receptors, which are G protein-coupled receptors that open ion channels via intermediaries known as G proteins (PMID: 10561820). Activation of the GABA-B receptor by GABA causes neuronal membrane hyperpolarization and a resultant inhibition of neurotransmitter release. In addition to binding sites for GABA, the GABA-A receptor has binding sites for benzodiazepines, barbiturates, and neurosteroids. GABA-A receptors are coupled to chloride ion channels. Therefore, activation of the GABA-A receptor induces increased inward chloride ion flux, resulting in membrane hyperpolarization and neuronal inhibition (PMID: 10561820). After release into the synapse, free GABA that does not bind to either the GABA-A or GABA-B receptor complexes can be taken up by neurons and glial cells. Four different GABA membrane transporter proteins (GAT-1, GAT-2, GAT-3, and BGT-1), which differ in their distribution in the CNS, are believed to mediate the uptake of synaptic GABA into neurons and glial cells. The GABA-A receptor subtype regulates neuronal excitability and rapid changes in fear arousal, such as anxiety, panic, and the acute stress response (PMID: 10561820). Drugs that stimulate GABA-A receptors, such as the benzodiazepines and barbiturates, have anxiolytic and anti-seizure effects via GABA-A-mediated reduction of neuronal excitability, which effectively raises the seizure threshold. GABA-A antagonists produce convulsions in animals and there is decreased GABA-A receptor binding in a positron emission tomography (PET) study of patients with panic disorder. Neurons that produce GABA as their output are called GABAergic neurons and have chiefly inhibitory action at receptors in the vertebrate. Medium spiny neurons (MSNs) are a typical example of inhibitory CNS GABAergic cells. GABA has been shown to have excitatory roles in the vertebrate, most notably in the developing cortex. Organisms synthesize GABA from glutamate using the enzyme L-glutamic acid decarboxylase and pyridoxal phosphate as a cofactor (PMID: 12467378). It is worth noting that this involves converting the principal excitatory neurotransmitter (glutamate) into the principal inhibitory one (GABA). Drugs that act as agonists of GABA receptors (known as GABA analogs or GABAergic drugs), or increase the available amount of GABA typically have relaxing, anti-anxiety, and anti-convulsive effects. GABA is found to be deficient in cerebrospinal fluid and the brain in many studies of experimental and human epilepsy. Benzodiazepines (such as Valium) are useful in status epilepticus because they act on GABA receptors. GABA increases in the brain after administration of many seizure medications. Hence, GABA is clearly an antiepileptic nutrient. Inhibitors of GAM metabolism can also produce convulsions. Spasticity and involuntary movement syndromes, such as Parkinson's, Friedreich's ataxia, tardive dyskinesia, and Huntington's chorea, are all marked by low GABA when amino acid levels are studied. Trials of 2 to 3 g of GABA given orally have been effective in various epilepsy and spasticity syndromes. Agents that elevate GABA are also useful in lowering hypertension. Three grams orally have been effective in controlling blood pressure. GABA is decreased in various encephalopathies. GABA can reduce appetite and is decreased in hypoglycemics. GABA reduces blood sugar in diabetics. Chronic brain syndromes can also be marked by deficiencies of GABA. Vitamin B6, manganese, taurine, and lysine can increase both GABA synthesis and effects, while aspartic acid and glutamic acid probably inhibit GABA effects. Low plasma GABA has been reported in some depressed patients and may be a useful trait marker for mood disorders. GABA has an important role in embryonic development, especially facial development, as substantiated by the association of a cleft palate in transgenic mice deficient in GAD67 (glutamate decarboxylase). A recent Japanese population study reported linkage in patients with a nonsyndromic cleft lip with or without a cleft palate and specific GAD67 haplotypes (PMID: 23842532). Unusually high levels of GABA (especially in the brain) can be toxic and GABA can function as both a neurotoxin and a metabotoxin. A neurotoxin is a compound that damages the brain and/or nerve tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of GABA are associated with at least five inborn errors of metabolism, including D-2-hydroxyglutaric aciduria, 4-hydroxybutyric aciduria/succinic semialdehyde dehydrogenase deficiency, GABA-transaminase deficiency, homocarnosinosis, and hyper beta-alaninemia. Nearly all of these conditions are associated with seizures, hypotonia, intellectual deficits, macrocephaly, encephalopathy, and other serious neurological or neuromuscular problems. Increased levels of GABA seem to alter the function of the GABA-B receptor, which may play a role in the tonic-clonic seizures that are often seen in patients with the above disorders.
56-12-2
C00334
119
16865
4-AMINO-BUTYRATE
116
DB02530
NCCCC(O)=O
C4H9NO2
InChI=1S/C4H9NO2/c5-3-1-2-4(6)7/h1-3,5H2,(H,6,7)
BTCSSZJGUNDROE-UHFFFAOYSA-N
4-aminobutanoic acid
103.1198
103.063328537
0.55
2
gamma(amino)-butyric acid
0
0
FDB008937
3-carboxypropylamine;4-aminobutanoate;4-aminobutanoic acid;4-aminobutyrate;4-aminobutyric acid;Aminalon;Gaba;Gaballon;Gamarex;Gammalon;Gammalone;Gammar;Gammasol;Mielogen;Mielomade;Omega-aminobutyrate;Omega-aminobutyric acid;Piperidate;Piperidic acid;Piperidinate;Piperidinic acid;Gamma aminobutyrate;Gamma aminobutyric acid;Gamma-aminobutyrate;Gamma-aminobutyric acid;W-aminobutyrate;W-aminobutyric acid;G-aminobutyrate;G-aminobutyric acid;γ-aminobutyrate;γ-aminobutyric acid
PW_C000071
GABA
120
1
145
8
15226
151
15227
220
15228
222
78013
111
80716
135
120189
122
125546
297
127079
205
972
Succinic acid semialdehyde
HMDB0001259
Succinic acid semialdehyde (or succinate semialdehyde) is an intermediate in the catabolism of gamma-aminobutyrate or GABA (PMID: 16435183). It is formed from GABA by the action of GABA transaminase, which leads to the production of succinate semialdehyde and alanine. The resulting succinate semialdehyde is further oxidized by succinate semialdehyde dehydrogenase to become succinic acid, which also yields NADPH. Under certain situations, high levels of succinate semialdehyde can function as a neurotoxin and a metabotoxin. A neurotoxin is a compound that causes damage to the brain and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Elevated serum levels of succinate semialdehyde are found in succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria), a rare neurometabolic disorder of gamma-aminobutyric acid (GABA) degradation. Symptoms include motor delay, hypotonia, speech delay, autistic features, seizures, and ataxia. Patients also exhibit behavioural problems such as attention deficit, hyperactivity, anxiety, or aggression (PMID: 18622364). Succinate semialdehyde is considered a reactive carbonyl and may lead to increased oxidative stress. This stress is believed to contribute to the formation of free radicals in the brain tissue of animal models induced with SSADH deficiency, which further leads to secondary cell damage and death. Additionally, oxidative stress may be responsible for the loss of striatal dopamine, which may contribute to the neuropathology of SSADH deficiency.
692-29-5
C00232
1112
16265
SUCC-S-ALD
1080
OC(=O)CCC=O
C4H6O3
InChI=1S/C4H6O3/c5-3-1-2-4(6)7/h3H,1-2H2,(H,6,7)
UIUJIQZEACWQSV-UHFFFAOYSA-N
4-oxobutanoic acid
102.0886
102.031694058
0.28
1
succinic semialdehyde
0
-1
FDB022516
2-formylpropionic acid ethyl ester;3-formylpropanoate;3-formylpropanoic acid;4-oxobutanoate;4-oxobutanoic acid;Butryaldehydate;Butryaldehydic acid;Succinaldehydate;Succinate semialdehyde;Succinic acid semialdehyde;Succinic semialdehyde;Beta-formylpropionate;Beta-formylpropionic acid;Gamma-oxybutyrate;Gamma-oxybutyric acid;3-formylpropionic acid;Semialdehyde succinique;Succinaldehydic acid;3-formylpropionate;B-formylpropionate;B-formylpropionic acid;β-formylpropionate;β-formylpropionic acid
PW_C000972
SuccASA
147
8
149
3
15229
222
15235
151
78014
111
80719
135
120190
122
125547
297
127080
205
174
Succinic acid
HMDB0000254
Succinic acid is a dicarboxylic acid. The anion, succinate, is a component of the citric acid cycle capable of donating electrons to the electron transfer chain. Succinate dehydrogenase (SDH) plays an important role in the mitochondria, being both part of the respiratory chain and the Krebs cycle. SDH with a covalently attached FAD prosthetic group, binds enzyme substrates (succinate and fumarate) and physiological regulators (oxaloacetate and ATP). Oxidizing succinate links SDH to the fast-cycling Krebs cycle portion where it participates in the breakdown of acetyl-CoA throughout the whole Krebs cycle. The succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate. (PMID 16143825) Mutations in the four genes encoding the subunits of the mitochondrial respiratory chain succinate dehydrogenase are associated with a wide spectrum of clinical presentations (i.e.: Huntington's disease. (PMID 11803021).
110-15-6
C00042
1110
15741
SUC
1078
DB00139
OC(=O)CCC(O)=O
C4H6O4
InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
KDYFGRWQOYBRFD-UHFFFAOYSA-N
butanedioic acid
118.088
118.02660868
0.25
2
succinic acid
0
-2
FDB001931
1,2-ethanedicarboxylate;1,2-ethanedicarboxylic acid;1,4-butanedioate;1,4-butanedioic acid;Amber acid;Asuccin;Dihydrofumarate;Dihydrofumaric acid;Katasuccin;Succinate;Wormwood acid;Acide butanedioique;Acide succinique;Acidum succinicum;Bernsteinsaeure;Butandisaeure;Butanedionic acid;E363;Ethylenesuccinic acid;Hooc-ch2-ch2-cooh;Spirit of amber;Butanedionate;Ethylenesuccinate
PW_C000174
Succini
152
3
239
4
502
18
507
8
676
31
1265
5
4255
17
5383
103
6042
155
6102
161
6454
107
6455
108
6489
178
6764
117
6836
166
7362
163
7455
219
7456
220
7477
222
11866
198
12142
151
13259
223
42368
318
42369
315
42402
322
77143
133
77213
134
77483
111
77738
112
77749
129
78426
334
80024
368
80721
119
112846
308
113428
1
119984
406
120192
407
120385
122
120555
414
120990
408
122565
384
122767
120
123029
135
123189
450
123555
374
125138
121
125364
479
125549
481
125930
482
126713
480
126906
501
127082
206
127389
502
128304
391
40034
Hydrogen Ion
HMDB0059597
Hydrogen ion is recommended by IUPAC as a general term for all ions of hydrogen and its isotopes. Depending on the charge of the ion, two different classes can be distinguished: positively charged ions and negatively charged ions. Under aqueous conditions found in biochemistry, hydrogen ions exist as the hydrated form hydronium, H3O+, but these are often still referred to as hydrogen ions or even protons by biochemists. [WikiPedia])
C00080
1038
15378
1010
[H+]
H
InChI=1S/p+1
GPRLSGONYQIRFK-UHFFFAOYSA-N
hydron
1.0079
1.007825032
0
hydron
1
0
H+;H(+);Hydrogen cation;Hydron;Proton
PW_C040034
H+
215
4
670
8
753
15
788
31
848
3
1116
2
1463
26
1464
54
2231
49
2780
17
4250
22
4254
42
4547
10
4576
18
4694
70
5241
103
5327
111
5353
112
5626
108
5639
107
5699
100
5720
105
5742
117
5963
147
6037
155
6070
157
6093
161
6130
159
6232
166
6483
178
6601
152
6692
101
6843
188
6910
187
7100
163
7168
205
7191
206
7453
219
7454
220
7472
222
7525
213
7532
210
7558
212
7572
160
7590
170
8195
225
8218
151
8243
226
8413
162
8420
224
9139
195
9155
249
11915
164
12015
281
12181
285
12246
286
12266
287
12521
227
13257
223
13325
294
15330
308
42329
315
42354
318
42401
322
42405
312
42454
320
76912
293
77136
133
77210
134
77372
331
77804
114
77955
132
77990
327
77991
347
78379
345
79929
130
80019
368
80387
310
80388
304
80722
119
93823
124
94823
383
110550
388
112855
94
113280
390
115537
398
115539
118
115856
336
116205
109
119973
406
120193
407
120549
122
120593
409
121170
424
121171
425
122569
418
122615
384
122687
125
122758
120
123183
135
123218
137
123742
459
123743
460
125141
454
125188
121
125273
136
125359
479
125550
481
125730
483
125736
297
125809
299
126517
495
126717
489
126766
480
126823
300
126902
501
127213
208
128308
506
128361
391
128430
395
9795
Zinc
HMDB0015532
Zinc is an essential element, necessary for sustaining all life. It is a trace element in the diet, forming an essential part of many enzymes, and playing an important role in protein synthesis and in cell division. Physiologically, it exists as an ion in the body. It is estimated that 3000 of the hundreds of thousands of proteins in the human body contain zinc prosthetic groups. In addition, there are over a dozen cell types in the human body that secrete zinc ions, and the roles of these secreted zinc signals in medicine and health are now being actively studied. Intriguingly, brain cells in the mammalian forebrain are one type of cell that secretes zinc, along with its other neuronal messenger substances. Cells in the salivary gland, prostate, immune system, and intestine are other types that secrete zinc. Obtaining a sufficient zinc intake during pregnancy and in young children is a problem, especially among those who cannot afford a good and varied diet. Zinc deficiency is associated with anemia, short stature, hypogonadism, impaired wound healing, and geophagia. Brain development is stunted by zinc deficiency in utero and in youth. Zinc is an activator of certain enzymes, such as carbonic anhydrase. Carbonic anhydrase is important in the transport of carbon dioxide in vertebrate blood. Even though zinc is an essential requirement for a healthy body, too much zinc can be harmful. Excessive absorption of zinc can also suppress copper and iron absorption. The free zinc ion in solution is highly toxic to plants, invertebrates, and even vertebrate fish. The Free Ion Activity Model (FIAM) is well-established in the literature and shows that just micromolar amounts of the free ion kill some organisms.
7440-66-6
23994
27363
22430
DB01593
[Zn]
Zn
InChI=1S/Zn
HCHKCACWOHOZIP-UHFFFAOYSA-N
zinc(2+) ion
65.409
63.929146578
0
zinc(2+) ion
2
2
30zn;Cinc;Zincum;Zink;Zn;Zn(ii);Zn2+
PW_C009795
Zinc
57
8
1711
2
1904
3
2137
17
2154
49
3610
29
4083
7
4469
18
4543
14
4999
31
6689
107
6690
101
6699
108
7020
160
11758
115
12229
151
12633
65
42397
315
42399
318
77030
253
78023
132
78328
112
78811
111
120119
124
120898
122
122308
407
122852
118
123469
135
124860
119
125486
299
126474
481
127023
388
127317
205
128043
206
8
tRNA(Glu)
RNA
PW_NA000008
29175
TRNAGLU
130
8
157
4
78010
111
79322
112
80702
135
120182
122
125539
297
127072
205
7
L-Glutamyl-tRNA(Glu)
RNA
PW_NA000007
29157
LGTG
129
8
158
4
78011
111
79321
112
80703
135
120183
122
125540
297
127073
205
41
tRNA(Gln)
RNA
945257
PW_NA000041
TRNAGLN
79303
111
132272
8
133830
122
133875
135
42
L-Glutaminyl-tRNA(Gln)
RNA
PW_NA000042
LGTG
79304
111
132273
8
133829
122
133874
135
7736
Unknown
Unknown
12
3.1.1.1, 3.1.1.56
5529
130
79334
111
79335
133
79336
112
79532
113
79948
329
79951
134
79967
331
81809
132
131707
115
131712
128
132238
523
132246
328
132274
336
132323
114
132364
332
132366
116
132572
326
132577
535
132581
538
132582
348
132583
539
132584
541
132863
362
133487
354
134882
404
15063
Glutaminase liver isoform, mitochondrial
Q3TMJ6
Plays an important role in the regulation of glutamine catabolism. Promotes mitochondrial respiration and increases ATP generation in cells by catalyzing the synthesis of glutamate and alpha-ketoglutarate. Increases cellular anti-oxidant function via NADH and glutathione production (By similarity).
Gls2
12
3.5.1.2
117819
133
132233
112
15067
Glutamine--fructose-6-phosphate aminotransferase [isomerizing] 1
Q99MJ4
Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins. Regulates the circadian expression of clock genes ARNTL/BMAL1 and CRY1.
Gfpt1
12
2.6.1.16
133285
111
13642
Glucosamine 6-phosphate N-acetyltransferase
Q9JK38
Gnpnat1
12
2.3.1.4
77293
111
79373
115
13640
N-acetyl-D-glucosamine kinase
Q9QZ08
Converts endogenous N-acetylglucosamine (GlcNAc), a major component of complex carbohydrates, from lysosomal degradation or nutritional sources into GlcNAc 6-phosphate. Involved in the N-glycolylneuraminic acid (Neu5Gc) degradation pathway. Also has ManNAc kinase activity.
Nagk
12
2.7.1.59
77287
111
79241
115
14999
CAD protein
B7ZN27
This protein is a "fusion" protein encoding four enzymatic activities of the pyrimidine pathway (GATase, CPSase, ATCase and DHOase).
Cad
12
6.3.5.5; 2.1.3.2; 3.5.2.3
132373
132
132875
111
15080
Carbamoyl-phosphate synthase [ammonia], mitochondrial
Q6NX75
Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Cps1
12
6.3.4.16
132310
133
133286
112
13904
Amidophosphoribosyltransferase
Q8CIH9
Ppat
12
2.4.2.14
79277
111
15085
GMP synthase [glutamine-hydrolyzing]
Q66JZ6
Involved in the de novo synthesis of guanine nucleotides which are not only essential for DNA and RNA synthesis, but also provide GTP, which is involved in a number of cellular processes important for cell division.
Gmps
12
6.3.5.2
133287
111
15090
Glutamine-dependent NAD(+) synthetase
Q9D280
Nadsyn1
12
6.3.5.1
133288
111
133449
132
15093
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Q8R1S2
Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes (By similarity).
Aldh4a1
12
1.2.1.88
133289
112
14085
Alanine aminotransferase 1
Q8QZR5
Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).
Gpt
12
2.6.1.2
79351
132
79375
111
79550
327
79908
133
15099
Glutamate dehydrogenase 1, mitochondrial
Q8C273
Mitochondrial glutamate dehydrogenase that converts L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate.
Glud1
12
1.4.1.3
117818
133
133290
112
133358
347
15103
Aspartate aminotransferase, mitochondrial
Q5HZH5
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
Got2
12
2.6.1.1; 2.6.1.7
132311
133
133291
111
133332
112
133333
345
13919
Probable glutamate--tRNA ligase, mitochondrial
Q9CXJ1
Catalyzes the attachment of glutamate to tRNA(Glu) in a two-step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu).
Ears2
12
6.1.1.17
79318
112
79378
111
15045
Glutamate--cysteine ligase catalytic subunit
Q8VCS5
Gclc
12
6.3.2.2
133282
132
133293
111
14135
Glutamate--cysteine ligase regulatory subunit
O09172
Gclm
12
79380
111
79558
132
13916
Glutathione synthetase
P51855
Gss
12
6.3.2.3
79313
111
15023
Glutathione reductase, mitochondrial
Q8C9Z6
Maintains high levels of reduced glutathione in the cytosol.
Gsr
12
1.8.1.7
133277
112
14960
Glutamate decarboxylase 1
O08685
Catalyzes the production of GABA.
Gad1
12
4.1.1.15
132395
111
14955
4-aminobutyrate aminotransferase, mitochondrial
Q8BZA3
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).
Abat
12
2.6.1.19; 2.6.1.22
132329
132
132353
112
133294
111
15114
Succinate-semialdehyde dehydrogenase, mitochondrial
Q5SZW1
Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Aldh5a1
12
1.2.1.24
133295
112
13912
Glutamine--tRNA ligase
Q8BML9
Glutamine--tRNA ligase. Plays a critical role in brain development.
Qars
12
6.1.1.18
79301
111
13926
Glutamine synthetase
P15105
Essential for proliferation of fetal skin fibroblasts. This enzyme has 2 functions: it catalyzes the production of glutamine and 4-aminobutanoate (gamma-aminobutyric acid, GABA), the latter in a pyridoxal phosphate-independent manner (By similarity).
Glul
12
6.3.1.2; 4.1.1.15
79337
133
79370
111
13655
Glutaminase kidney isoform, mitochondrial
D3Z7P3
Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine. Plays a role in maintaining acid-base homeostasis. Regulates the levels of the neurotransmitter glutamate in the brain.
Gls
12
3.5.1.2
77335
133
79371
112
13644
Glutamine--fructose-6-phosphate aminotransferase [isomerizing] 2
Q9Z2Z9
Controls the flux of glucose into the hexosamine pathway. Most likely involved in regulating the availability of precursors for N- and O-linked glycosylation of proteins.
Gfpt2
12
2.6.1.16
77297
111
7797
CAD protein
B2RQC6
This protein is a "fusion" protein encoding four enzymatic activities of the pyrimidine pathway (GATase, CPSase, ATCase and DHOase).
Cad
12
6.3.5.5; 2.1.3.2; 3.5.2.3
5577
133
79283
111
79300
112
79738
132
13900
GMP synthase [glutamine-hydrolyzing]
Q3THK7
Involved in the de novo synthesis of guanine nucleotides which are not only essential for DNA and RNA synthesis, but also provide GTP, which is involved in a number of cellular processes important for cell division.
Gmps
12
6.3.5.2
79256
111
13898
Glutamine-dependent NAD(+) synthetase
Q711T7
Nadsyn1
12
6.3.5.1
79252
111
79651
132
13915
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Q8CHT0
Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes (By similarity).
Aldh4a1
12
1.2.1.88
79311
112
79431
133
7796
Probable inactive 1-aminocyclopropane-1-carboxylate synthase-like protein 2
Q3UX83
Accsl
12
5568
132
77974
346
78467
111
13654
Glutamate dehydrogenase 1, mitochondrial
P26443
Mitochondrial glutamate dehydrogenase that converts L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate.
Glud1
12
1.4.1.3
77334
133
77987
347
79376
112
13913
Aspartate aminotransferase, mitochondrial
P05202
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
Got2
12
2.6.1.1; 2.6.1.7
79305
112
79377
111
79626
345
79909
133
14134
Glutamate--cysteine ligase catalytic subunit
P97494
Gclc
12
6.3.2.2
79379
111
79557
132
13920
Glutamate decarboxylase 1
P48318
Catalyzes the production of GABA.
Gad1
12
4.1.1.15
79323
111
13921
4-aminobutyrate aminotransferase, mitochondrial
P61922
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine (By similarity).
Abat
12
2.6.1.19; 2.6.1.22
79325
133
79381
111
79445
112
79726
132
7800
1-aminocyclopropane-1-carboxylate synthase-like protein 1
A2AIG8
Does not catalyze the synthesis of 1-aminocyclopropane-1-carboxylate but is capable of catalyzing the deamination of L-vinylglycine.
Accs
12
7389
Glutamine synthetase
12
PW_P007389
15252
13926
10
6768
Glutaminase liver isoform, mitochondrial
12
PW_P006768
14579
13655
1
6750
Glutamine--fructose-6-phosphate aminotransferase
12
PW_P006750
14561
13644
4
6748
Glucosamine 6-phosphate N-acetyltransferase
12
PW_P006748
14559
13642
2
6746
N-acetyl-D-glucosamine kinase
12
PW_P006746
14557
13640
2
7365
CAD protein
12
PW_P007365
15227
7797
779
9795
780
423
79285
111
79286
128
6765
Carbamoyl-phosphate synthase [ammonia], mitochondrial
12
PW_P006765
14576
7797
1
7362
Amidophosphoribosyltransferase
12
PW_P007362
15224
13904
776
40711
79278
111
7360
GMP synthase [glutamine-hydrolyzing]
12
PW_P007360
15222
13900
79269
111
7355
Glutamine-dependent NAD(+) synthetase
12
PW_P007355
15217
13898
6
79253
111
7373
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
12
PW_P007373
15236
13915
79312
112
1337
Alanine aminotransferase 1
12
PW_P001337
1560
7796
2
6767
Glutamate dehydrogenase 1, mitochondrial
12
PW_P006767
14578
13654
6
7371
Aspartate aminotransferase, mitochondrial
12
PW_P007371
15234
13913
783
1148
79306
112
7377
Probable glutamate--tRNA ligase, mitochondrial
12
PW_P007377
15240
13919
79319
112
7401
Glutamate--cysteine ligase
12
PW_P007401
15265
14134
1
15266
14135
1
7374
Glutathione synthetase
12
PW_P007374
15237
13916
784
423
79314
111
7376
Glutathione reductase, mitochondrial
12
PW_P007376
785
964
79317
112
7378
Glutamate decarboxylase 1
12
PW_P007378
15241
13920
786
1148
79324
111
7379
4-aminobutyrate aminotransferase, mitochondrial
12
PW_P007379
15242
13921
787
1148
79326
133
7380
Succinate-semialdehyde dehydrogenase, mitochondrial
12
PW_P007380
79328
112
7370
Glutamine--tRNA ligase
12
PW_P007370
15233
13912
79302
111
1338
Alanine aminotransferase 2
12
PW_P001338
1561
7800
2
109664
false
PW_R109664
Right
398497
414
1
Compound
true
398498
95
1
Compound
false
398499
35
1
Compound
true
398500
1034
1
Compound
true
398501
1104
1
Compound
true
398502
500
1
Compound
false
99924
7389
6.3.1.2
108572
false
PW_R108572
Right
393968
500
1
Compound
false
393969
1420
1
Compound
true
393970
95
1
Compound
false
393971
35
1
Compound
true
98729
6768
3.5.1.2
108552
false
PW_R108552
Right
393882
79
1
Compound
false
393883
500
1
Compound
false
393884
969
1
Compound
false
393885
95
1
Compound
false
98709
6750
2.6.1.16
108550
false
PW_R108550
Right
393874
940
1
Compound
true
393875
969
1
Compound
false
393876
837
1
Compound
false
393877
1099
1
Compound
true
98707
6748
2.3.1.4
108855
false
PW_R108855
Right
395121
837
3
Compound
false
395122
914
3
Compound
true
395123
1858
3
Compound
false
395124
414
3
Compound
true
99038
6746
2.7.1.59
109688
false
PW_R109688
Right
398594
414
2
Compound
true
398595
500
1
Compound
false
398596
1916
1
Compound
true
398597
1420
1
Compound
true
398598
1034
2
Compound
true
398599
1441
1
Compound
true
398600
95
1
Compound
true
398601
862
1
Compound
false
99961
7365
108569
false
PW_R108569
Right
393949
414
2
Compound
true
393950
35
1
Compound
false
393951
1316
1
Compound
true
393952
1420
1
Compound
true
393953
1034
2
Compound
true
393954
1441
1
Compound
true
393955
862
1
Compound
false
98726
6765
6.3.4.16
109621
PW_R109621
Both
398292
882
1
Compound
false
398293
170
1
Compound
true
398294
95
1
Compound
true
398295
190
1
Compound
false
398296
500
1
Compound
true
398297
1420
1
Compound
true
99848
7362
2.4.2.14
109617
PW_R109617
Right
398272
414
1
Compound
true
398273
1420
1
Compound
true
398274
500
1
Compound
false
398275
1196
1
Compound
false
398276
32
1
Compound
true
398277
170
1
Compound
true
398278
95
1
Compound
true
398279
1080
1
Compound
true
99841
7360
109614
PW_R109614
Right
398256
915
1
Compound
false
398257
414
1
Compound
true
398258
1420
1
Compound
true
398259
500
1
Compound
false
398260
721
1
Compound
true
398261
32
1
Compound
true
398262
170
1
Compound
true
398263
95
1
Compound
true
99835
7355
109639
PW_R109639
Both
398370
1420
2
Compound
true
398371
721
1
Compound
true
398372
1003
1
Compound
false
398373
95
1
Compound
false
398374
1144
1
Compound
true
99878
7373
2627
false
PW_R002627
Both
10269
105
1
Compound
false
10270
134
1
Compound
true
10271
95
1
Compound
true
10272
164
1
Compound
false
2616
1337
2.6.1.2
2617
1338
2.6.1.2
108571
false
PW_R108571
Right
393962
95
1
Compound
false
393963
1420
1
Compound
true
393964
721
1
Compound
true
393965
134
1
Compound
false
393966
35
1
Compound
true
393967
1144
1
Compound
true
98728
6767
1.4.1.3
109689
false
PW_R109689
Both
398602
123
1
Compound
false
398603
134
1
Compound
false
398604
148
1
Compound
true
398605
95
1
Compound
false
99962
7371
109690
false
PW_R109690
Right
398606
134
1
Compound
false
398607
414
1
Compound
true
398608
8
1
NucleicAcid
true
398609
7
1
NucleicAcid
false
398610
32
1
Compound
true
398611
170
1
Compound
true
99963
7377
6.1.1.17
109691
false
PW_R109691
Right
398612
95
1
Compound
false
398613
414
1
Compound
true
398614
448
1
Compound
false
398615
826
1
Compound
false
398616
914
1
Compound
true
398617
1104
1
Compound
true
99964
7401
6.3.2.2
109641
PW_R109641
Right
398380
826
1
Compound
false
398381
414
1
Compound
true
398382
78
1
Compound
false
398383
80
1
Compound
false
398384
1034
1
Compound
true
398385
1104
1
Compound
true
99881
7374
109692
false
PW_R109692
Both
398618
1847
1
Compound
false
398619
146
1
Compound
true
398620
80
2
Compound
false
398621
143
1
Compound
true
99965
7376
1.8.1.7
109647
PW_R109647
Right
398411
95
1
Compound
false
398412
71
1
Compound
false
398413
1316
1
Compound
true
99893
7378
109649
PW_R109649
Both
398419
71
1
Compound
false
398420
134
1
Compound
false
398421
972
1
Compound
false
398422
95
1
Compound
false
99897
7379
109693
false
PW_R109693
Right
398622
972
1
Compound
false
398623
721
1
Compound
true
398624
1420
1
Compound
true
398625
174
1
Compound
false
398626
1144
1
Compound
true
398627
40034
1
Compound
true
99966
7380
1.2.1.24
108868
false
PW_R108868
Right
395194
500
1
Compound
false
395195
414
1
Compound
true
395196
41
1
NucleicAcid
true
395197
32
1
Compound
true
395198
170
1
Compound
true
395199
42
1
NucleicAcid
false
99051
7370
2650037
414
111
42
false
1717
1380
10
regular
50
30
2650038
95
111
81
false
1527
1445
10
regular
200
190
2650039
35
111
63
false
1457
1355
10
regular
78
78
2650040
1034
111
43
false
1689
1135
10
regular
50
30
2650041
1104
111
46
false
1554
1128
10
regular
44
43
2650042
500
111
81
false
1529
900
10
regular
200
190
2650043
20
111
9
false
1649
1212
10
regular
100
25
2650044
423
111
9
false
1504
1212
10
regular
100
25
2650045
1420
112
49
false
1934
1110
10
regular
78
78
2650046
35
112
63
false
1935
1346
10
regular
78
78
2650047
79
111
81
false
1744
780
10
regular
200
190
2650048
969
111
81
false
1529
430
10
regular
200
190
2650049
95
111
81
false
1734
560
10
regular
200
190
2650050
940
115
81
false
1949
500
10
regular
200
190
2650051
837
115
81
false
1524
70
10
regular
200
200
2650052
1099
115
85
false
2089
185
10
regular
50
30
2650053
914
111
43
false
1444
220
10
regular
50
30
2650054
1858
111
81
false
934
80
10
regular
200
190
2650055
414
111
42
false
1164
220
10
regular
50
30
2650056
414
111
42
false
1169
1490
10
regular
50
30
2650057
1916
111
3
false
1164
1270
10
regular
100
100
2650058
1420
111
49
false
1319
1405
10
regular
78
78
2650059
1034
111
43
false
934
1488
10
regular
50
30
2650060
1441
111
46
false
877
1486
10
regular
44
43
2650061
95
111
3
false
894
1292
10
regular
100
110
2650062
862
111
81
false
649
1352
10
regular
200
190
2650063
1005
111
9
false
1029
1395
10
regular
100
25
2650064
414
112
42
false
319
1367
10
regular
50
30
2650065
35
112
63
false
160
1413
10
regular
78
78
2650066
1316
112
52
false
225
1316
10
regular
78
78
2650067
1420
112
49
false
234
1498
10
regular
78
78
2650068
1034
112
43
false
574
1364
10
regular
50
30
2650069
1441
112
46
false
572
1497
10
regular
44
43
2650070
882
111
81
false
554
1043
10
regular
200
190
2650071
170
111
45
false
857
1191
10
regular
63
43
2650072
95
111
81
false
764
901
10
regular
200
190
2650073
190
111
81
false
1114
900
10
regular
200
190
2650074
1420
111
49
false
1179
1180
10
regular
78
78
2650075
423
111
9
false
1014
1163
10
regular
100
25
2650076
40711
111
9
false
1019
1088
10
regular
100
25
2650077
414
111
42
false
959
845
10
regular
50
30
2650078
1196
111
81
false
974
535
10
regular
200
190
2650079
1420
111
49
false
1034
825
10
regular
78
78
2650080
32
111
44
false
729
843
10
regular
50
30
2650081
170
111
45
false
642
836
10
regular
63
43
2650082
95
111
81
false
604
536
10
regular
200
190
2650083
1080
111
81
false
379
696
10
regular
200
190
2650084
414
111
42
false
1214
285
10
regular
50
30
2650085
915
111
81
false
1309
275
10
regular
200
200
2650086
1420
111
49
false
1199
440
10
regular
78
78
2650087
95
111
3
false
864
428
10
regular
100
110
2650088
32
111
44
false
944
283
10
regular
50
30
2650089
170
111
45
false
842
276
10
regular
63
43
2650090
721
111
81
false
569
281
10
regular
200
190
2650091
1003
112
3
false
1352
2120
10
regular
100
120
2650092
721
112
59
false
1292
2055
10
regular
50
30
2650093
1420
112
49
false
1357
1765
10
regular
78
78
2650094
1144
112
60
false
1272
1880
10
regular
50
30
2650095
105
111
3
false
1627
1905
10
regular
100
100
2650096
134
111
81
false
1482
2100
10
regular
200
190
2650097
164
111
3
false
1632
1715
10
regular
100
100
2650098
1148
111
9
false
2077
1847
10
regular
100
35
2650099
1420
112
49
false
1792
1645
10
regular
78
78
2650100
721
112
59
false
1867
1760
10
regular
50
30
2650101
35
112
63
false
1790
1981
10
regular
78
78
2650102
1144
112
60
false
1872
1915
10
regular
50
30
2650103
123
111
3
false
2002
2000
10
regular
100
110
2650104
148
111
3
false
2002
1660
10
regular
100
110
2650105
1148
111
9
false
1462
1812
10
regular
100
35
2650106
414
111
42
false
1327
2255
10
regular
50
30
2650107
32
111
44
false
1097
2258
10
regular
50
30
2650108
170
111
45
false
1085
2396
10
regular
63
43
2650109
414
111
42
false
932
1910
10
regular
50
30
2650110
448
111
81
false
1042
1915
10
regular
200
190
2650111
826
111
81
false
437
1923
10
regular
200
190
2650112
914
111
43
false
672
1913
10
regular
50
30
2650113
1104
111
46
false
670
2051
10
regular
44
43
2650114
414
111
42
false
422
2188
10
regular
50
30
2650115
78
111
81
false
652
2108
10
regular
200
190
2650116
80
111
81
false
436
2423
10
regular
200
190
2650117
1034
111
43
false
431
2368
10
regular
50
30
2650118
1104
111
46
false
599
2356
10
regular
44
43
2650119
423
111
9
false
392
2258
10
regular
100
25
2650120
1847
112
81
false
436
2903
10
regular
200
190
2650121
146
112
62
false
391
2823
10
regular
50
30
2650122
143
112
61
false
391
2653
10
regular
50
30
2650123
964
112
9
false
396
2723
10
regular
100
25
2650124
71
111
81
false
1490
2630
10
regular
200
190
2650125
1316
111
52
false
1451
2531
10
regular
78
78
2650126
1148
111
9
false
1472
2425
10
regular
100
35
2650127
134
111
81
false
1715
2720
10
regular
200
190
2650128
972
111
81
false
1490
3020
10
regular
200
190
2650129
95
111
81
false
1715
2935
10
regular
200
190
2650130
1148
111
9
false
1450
2880
10
regular
100
35
2650131
721
112
59
false
1430
3155
10
regular
50
30
2650132
1420
112
49
false
1400
2995
10
regular
78
78
2650133
174
112
81
false
945
3018
10
regular
200
190
2650134
1144
112
60
false
1175
3153
10
regular
50
30
2650135
40034
112
55
false
1161
3004
10
regular
78
78
2650136
414
42
false
1174
1580
10
regular
50
30
2650137
32
44
false
824
1568
10
regular
50
30
2650138
170
45
false
732
1653
10
regular
63
43
3414
8
12
111
false
1387
2305
14
150
nucleic_acid
regular
150
70
3415
7
12
111
false
912
2306
14
150
nucleic_acid
regular
150
70
3416
41
12
false
1129
1740
14
150
nucleic_acid
regular
150
70
3417
42
12
false
774
1761
14
150
nucleic_acid
regular
150
70
922563
7736
111
136
false
1539
1187
8
protein
regular
175
155
922564
15063
112
2
false
1809
1232
8
subunit
regular
150
70
922565
15067
111
8
false
1559
732
8
subunit
regular
140
85
922566
13642
115
6
false
2114
312
8
subunit
regular
160
80
922567
13640
111
6
false
1249
130
8
subunit
regular
160
80
922568
14999
111
17
false
984
1405
8
subunit
regular
180
85
922569
15080
112
2
false
369
1412
8
subunit
regular
150
70
922570
13904
111
2
false
979
1103
8
subunit
regular
150
70
922571
15085
111
6
false
794
745
8
subunit
regular
160
80
922572
15090
111
17
false
999
335
8
subunit
regular
180
85
922573
15093
112
6
false
1322
1950
8
subunit
regular
160
80
922574
14085
111
6
false
1502
1827
8
subunit
regular
160
80
922575
15099
112
17
false
1742
1812
8
subunit
regular
180
85
922576
15103
111
6
false
1977
1847
8
subunit
regular
160
80
922577
13919
111
2
false
1167
2305
8
subunit
regular
150
70
922578
15045
111
113
false
747
1945
8
none
regular
160
80
922579
14135
111
6
false
752
1975
8
protein
regular
160
80
922580
13916
111
6
false
457
2238
8
subunit
regular
160
80
922581
15023
112
6
false
456
2718
8
subunit
regular
160
80
922582
14960
111
6
false
1512
2430
8
subunit
regular
160
80
922583
14955
111
6
false
1510
2880
8
subunit
regular
160
80
922584
15114
112
8
false
1245
3070
8
subunit
regular
140
85
922585
13912
111
2
false
941
1640
8
subunit
regular
150
70
790638
7389
64311
111
919074
922563
31145
2650043
3607652
Cofactor
31146
2650044
3607653
Cofactor
790639
6768
64311
112
919075
922564
790640
6750
64311
111
919076
922565
790641
6748
64311
115
919077
922566
790642
6746
64311
111
919078
922567
790643
7365
64311
111
919079
922568
31147
2650063
3607678
Cofactor
790644
6765
64311
112
919080
922569
790645
7362
64311
111
919081
922570
31148
2650075
3607692
Cofactor
31149
2650076
3607693
Cofactor
790646
7360
64311
111
919082
922571
790647
7355
64311
111
919083
922572
790648
7373
64311
112
919084
922573
790649
1337
64311
111
919085
922574
31150
2650098
3607719
Cofactor
790650
6767
64311
112
919086
922575
790651
7371
64311
111
919087
922576
31151
2650105
3607730
Cofactor
790652
7377
64311
111
919088
922577
790653
7401
64311
111
919089
922578
919090
922579
790654
7374
64311
111
919091
922580
31152
2650119
3607749
Cofactor
790655
7376
64311
112
919092
922581
31153
2650123
3607754
Cofactor
790656
7378
64311
111
919093
922582
31154
2650126
3607758
Cofactor
790657
7379
64311
111
919094
922583
31155
2650130
3607763
Cofactor
790658
7380
64311
112
919095
922584
790659
7370
64311
919096
922585
3607646
M1717 1395 C1690 1395 1629 1372 1629 1342
5
false
18
false
false
3607647
M1627 1445 C1627 1415 1629 1372 1629 1342
5
false
18
false
false
3607648
M1535 1394 C1563 1393 1629 1372 1629 1342
5
false
18
false
false
3607649
M1689 1150 C1660 1150 1629 1164 1629 1194
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607650
M1598 1149.5 C1630 1150.5 1629 1164 1629 1194
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607651
M1629 1090 C1629 1120 1629 1164 1629 1194
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607652
M1045 1097 L1045 1147 L1095 1097 z
10
true
18
3607653
M1045 1097 L1045 1147 L1095 1097 z
10
true
18
3607654
M1729 995 C1901 1063 1879 1189 1884 1232
5
false
18
true
M 1238.8379857931677 447.8013236705336 L 1224 450 L 1233.3231024526012 461.75073447314253
false
3607655
M1934 1149 C1887 1156 1886 1206 1884 1232
5
false
18
true
M 1422.289154082854 608.3731578406037 L 1434 599 L 1420.027184237783 593.5446888562483
false
3607656
M1727 1455 C1900 1447 1882 1338 1884 1302
5
false
18
false
false
3607657
M1935 1385 C1887 1389 1895 1346 1884 1302
5
false
18
false
false
3607658
M1744 875 C1654 877 1629 847 1629 817
5
false
18
3607659
M1629 900 C1629 870 1629 847 1629 817
5
false
18
3607660
M1629 620 C1629 650 1629 702 1629 732
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607661
M1734 655 C1629 663 1629 702 1629 732
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607662
M2149 595 C2177 584 2194 422 2194 392
5
false
18
3607663
M1729 525 C1877 514 2210 480 2194 392
5
false
18
3607664
M1724 170 C1903 167 2194 235 2194 312
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607665
M2139 200 C2175 242 2194 282 2194 312
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607666
M1524 170 C1494 170 1439 170 1409 170
5
false
18
3607667
M1469 220 C1462 180 1439 170 1409 170
5
false
18
3607668
M1134 175 C1164 175 1219 170 1249 170
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607669
M1189 220 C1183 189 1219 170 1249 170
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607670
M1194 1490 C1192 1460 1187 1445 1164 1445
5
false
18
3607671
M1529 1068 C1388 1176 1278 1448 1164 1445
5
false
18
3607672
M1214 1370 C1215 1430 1194 1445 1164 1445
5
false
18
3607673
M1319 1444 C1270 1445 1194 1445 1164 1445
5
false
18
3607674
M959 1488 C963 1464 954 1445 984 1445
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607675
M899 1486 C902 1437 954 1445 984 1445
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607676
M944 1402 C948 1431 954 1445 984 1445
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607677
M849 1447 C879 1447 954 1445 984 1445
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607678
M1194 910 L1194 960 L1244 910 z
10
true
18
3607679
M344 1397 C343 1424 339 1447 369 1447
5
false
18
true
M 216.99038105676658 1274.5 L 204 1282 L 216.99038105676658 1289.5
false
3607680
M238 1452 C310 1450 339 1447 369 1447
5
false
18
true
M 271.7392356964738 1233.8492891178628 L 264 1221 L 256.7418070515968 1234.127019277953
false
3607681
M264 1394 C296 1454 339 1447 369 1447
5
false
18
true
M 210.2898205670534 1336.4660890130929 L 214 1351 L 224.73164641453005 1340.5199348650162
false
3607682
M273 1498 C276 1468 339 1447 369 1447
5
false
18
true
M 296.8298123000733 1339.3278098408605 L 303 1353 L 311.7553701532231 1340.8203656261753
false
3607683
M599 1394 C596 1445 549 1447 519 1447
5
false
18
false
false
3607684
M594 1497 C597 1463 549 1447 519 1447
5
false
18
false
false
3607685
M649 1447 C619 1447 549 1447 519 1447
5
false
18
false
false
3607686
M754 1138 C784 1138 949 1138 979 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607687
M888.5 1191 C892.5 1146 949 1138 979 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607688
M864 1091 C881 1148 949 1138 979 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607689
M1529 995 C1468 1054 1330 1138 1129 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607690
M1214 1090 C1201 1144 1159 1138 1129 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607691
M1218 1180 C1215 1126 1159 1138 1129 1138
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607692
M150 150 L150 200 L200 150 z
10
true
18
3607693
M150 150 L150 200 L200 150 z
10
true
18
3607694
M984 845 C989 816 984 785 954 785
5
false
18
3607695
M1074 725 C1073 770 984 785 954 785
5
false
18
3607696
M1073 825 C1076 791 984 785 954 785
5
false
18
3607697
M1534 905 C1247 776 984 785 954 785
5
false
18
3607698
M754 843 C750 822 764 785 794 785
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607699
M673.5 836 C682.5 796 764 785 794 785
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607700
M704 726 C704 776 764 785 794 785
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607701
M579 791 C609 791 764 785 794 785
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607702
M1546 899 C1476 709 1311 369 1179 375
5
false
18
3607703
M1239 315 C1237 341 1209 375 1179 375
5
false
18
3607704
M1309 375 C1279 375 1209 375 1179 375
5
false
18
3607705
M1238 440 C1219 392 1209 375 1179 375
5
false
18
3607706
M914 428 C932 392 969 375 999 375
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607707
M969 313 C977 340 969 375 999 375
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607708
M873.5 319 C898.5 350 969 375 999 375
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607709
M769 376 C799 376 969 375 999 375
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607710
M1402 2120 C1402 2097 1400 2080 1402 2030
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607711
M1342 2070 C1373 2067 1406 2070 1402 2030
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607712
M1396 1843 C1396 1873 1402 1920 1402 1950
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607713
M1547 1637 C1513 1669 1403 1859 1402 1950
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607714
M1322 1895 C1358 1893 1403 1914 1402 1950
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607715
M1627 1955 C1589 1954 1582 1937 1582 1907
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607716
M1582 2100 C1582 2070 1577 1937 1577 1907
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607717
M1579 1635 C1579 1665 1582 1797 1582 1827
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607718
M1632 1765 C1581 1772 1582 1797 1582 1827
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607719
M1602 1772.5 L1602 1822.5 L1652 1772.5 z
10
true
18
3607720
M1712 1630 C1778 1653 1832 1782 1832 1812
5
false
18
3607721
M1831 1723 C1831 1753 1832 1782 1832 1812
5
false
18
3607722
M1832 1812 C1834 1788 1829 1778 1867 1775
5
false
18
3607723
M1669 2100 C1750 2072 1832 1927 1832 1897
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607724
M1829 1981 C1829 1951 1832 1927 1832 1897
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607725
M1872 1930 C1845 1938 1832 1927 1832 1897
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607726
M2052 2000 C2052 1970 2057 1957 2057 1927
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607727
M1682 2195 C1868 2175 2057 1957 2057 1927
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607728
M2052 1770 C2052 1800 2057 1817 2057 1847
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607729
M1730 1600 C1977 1655 2057 1817 2057 1847
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607730
M1557 1917.5 L1557 1967.5 L1607 1917.5 z
10
true
18
3607731
M1482 2195 C1448 2286 1347 2340 1317 2340
5
false
18
3607732
M1352 2285 C1347 2326 1335 2341 1317 2340
5
false
18
3607733
M1122 2288 C1134 2329 1137 2340 1167 2340
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607734
M1116.5 2396 C1118.5 2363 1137 2340 1167 2340
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607735
M1387 2340 C1357 2340 1347 2340 1317 2340
5
false
18
3607736
M1062 2341 C1092 2341 1137 2340 1167 2340
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false
18
true
M 1074.9903810567666 2333.5 L 1062 2341 L 1074.9903810567666 2348.5
false
3607737
M1526 1627 C1352 1773 1072 1948 912 2015
5
false
18
3607738
M957 1940 C963 2000 941 2012 912 2015
5
false
18
3607739
M1042 2010 C1012 2010 942 2015 912 2015
5
false
18
3607740
M637 2018 C667 2018 722 2015 752 2015
5
false
18
true
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false
3607741
M697 1943 C692 1999 722 2015 752 2015
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607742
M692 2051 C694 2020 722 2015 752 2015
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
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3607743
M537 2113 C537 2143 537 2208 537 2238
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18
3607744
M472 2203 C496 2201 537 2208 537 2238
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false
18
3607745
M652 2203 C606 2207 537 2208 537 2238
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3607746
M536 2423 C536 2393 537 2348 537 2318
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18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
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3607747
M481 2383 C511 2373 537 2348 537 2318
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true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607748
M599 2377.5 C560 2371.5 537 2348 537 2318
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true
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false
3607749
M447 2248 L447 2298 L497 2248 z
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true
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3607750
M536 2903 C534 2869 537 2834 536 2798
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607751
M441 2838 C488 2840 543 2835 536 2798
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
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3607752
M536 2613 C535 2664 535 2679 536 2718
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false
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true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607753
M441 2668 C510 2665 535 2679 536 2718
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true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607754
M150 150 L150 200 L200 150 z
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true
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3607755
M1727 1586 C2192 1657 2201 1680 2201 1727 C2233 2442 1596 2296 1592 2430
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false
18
false
false
3607756
M1590 2630 C1590 2600 1592 2540 1592 2510
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607757
M1529 2570 C1586 2570 1592 2540 1592 2510
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607758
M1712 1770 L1712 1820 L1762 1770 z
10
true
18
3607759
M1590 2820 C1590 2850 1590 2850 1590 2880
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607760
M1715 2815 C1667 2825 1590 2841 1590 2880
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
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3607761
M1590 3020 C1590 2990 1590 2990 1590 2960
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607762
M1715 3030 C1651 3007 1590 2990 1590 2960
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607763
M1565 2955 L1565 3005 L1615 2955 z
10
true
18
3607764
M1490 3115 C1460 3115 1415 3115 1385 3115
5
false
18
3607765
M1455 3155 C1447 3134 1415 3115 1385 3115
5
false
18
3607766
M1439 3073 C1434 3094 1415 3115 1385 3115
5
false
18
3607767
M1145 3113 C1175 3113 1215 3115 1245 3115
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607768
M1200 3153 C1203 3116 1215 3115 1245 3115
5
false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607769
M1200 3082 C1206 3106 1215 3115 1245 3115
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false
18
true
M 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345
false
3607770
M1533 1089 C1477 1215 1442 1687 1091 1675
5
false
18
3607771
M1199 1610 C1199 1657 1138 1676 1091 1675
5
false
18
3607772
M849 1598 C848 1635 915 1677 941 1675
5
false
18
true
M 851.9468550441649 1030.261556296296 L 837 1029 L 843.3808877211858 1042.5751343230784
false
3607773
M795 1674.5 C827 1673.5 907 1675 941 1675
5
false
18
true
M 851.9468550441649 1030.261556296296 L 837 1029 L 843.3808877211858 1042.5751343230784
false
3607774
M1204 1740 C1205 1702 1128 1677 1091 1675
5
false
18
3607775
M849 1761 C850 1719 904 1675 941 1675
5
false
18
true
M 851.9468550441649 1030.261556296296 L 837 1029 L 843.3808877211858 1042.5751343230784
false
3607776
M1727 1540 C1790 1542 2258 1540 2302 1540
5
false
18
false
false
true
M 2284.0096189432334 1536.5 L 2297 1529 L 2284.0096189432334 1521.5
false
3607777
M749 1542 C749 1582 703 1640 629 1667
5
false
18
true
M 598.632736368666 1490.501722300632 L 589 1502 L 603.7741687717536 1504.593055553539
false
3607778
M554 1138 C516 1139 434 1137 394 1138
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false
18
true
M 521.7988820308822 990.1776845653245 L 509 998 L 522.1737648982852 1005.1729992614463
false
3607779
M1728 1567 C1793 1576 2239 1635 2297 1645
5
false
18
true
M 1741.8962681635894 1561.352546491948 L 1728 1567 L 1739.8389722864595 1576.2107944934435
false
3607780
M1726 1487 C1756 1487 2272 1400 2302 1400
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false
18
true
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false
3607781
M1490 2725 C1460 2725 1425 2725 1395 2725
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false
18
true
M 1407.9903810567666 2717.5 L 1395 2725 L 1407.9903810567666 2732.5
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3607782
M1352 2180 C1307 2181 1256 2180 1217 2180
5
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18
true
M 1229.9903810567666 2172.5 L 1217 2180 L 1229.9903810567666 2187.5
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788651
64311
109664
111
3192832
2650037
3607646
Left
3192833
2650038
3607647
Left
3192834
2650039
3607648
Left
3192835
2650040
3607649
Right
3192836
2650041
3607650
Right
3192837
2650042
3607651
Right
745300
99924
790638
788652
64311
108572
112
3192838
2650042
3607654
Left
3192839
2650045
3607655
Left
3192840
2650038
3607656
Right
3192841
2650046
3607657
Right
745301
98729
790639
788653
64311
108552
111
3192842
2650047
3607658
Left
3192843
2650042
3607659
Left
3192844
2650048
3607660
Right
3192845
2650049
3607661
Right
745302
98709
790640
788654
64311
108550
115
3192846
2650050
3607662
Left
3192847
2650048
3607663
Left
3192848
2650051
3607664
Right
3192849
2650052
3607665
Right
745303
98707
790641
788655
64311
108855
111
3192850
2650051
3607666
Left
3192851
2650053
3607667
Left
3192852
2650054
3607668
Right
3192853
2650055
3607669
Right
745304
99038
790642
788656
64311
109688
111
3192854
2650056
3607670
Left
3192855
2650042
3607671
Left
3192856
2650057
3607672
Left
3192857
2650058
3607673
Left
3192858
2650059
3607674
Right
3192859
2650060
3607675
Right
3192860
2650061
3607676
Right
3192861
2650062
3607677
Right
745305
99961
790643
788657
64311
108569
112
3192862
2650064
3607679
Left
3192863
2650065
3607680
Left
3192864
2650066
3607681
Left
3192865
2650067
3607682
Left
3192866
2650068
3607683
Right
3192867
2650069
3607684
Right
3192868
2650062
3607685
Right
745306
98726
790644
788658
64311
109621
111
3192869
2650070
3607686
Left
3192870
2650071
3607687
Left
3192871
2650072
3607688
Left
3192872
2650042
3607689
Right
3192873
2650073
3607690
Right
3192874
2650074
3607691
Right
745307
99848
790645
788659
64311
109617
111
3192875
2650077
3607694
Left
3192876
2650078
3607695
Left
3192877
2650079
3607696
Left
3192878
2650042
3607697
Left
3192879
2650080
3607698
Right
3192880
2650081
3607699
Right
3192881
2650082
3607700
Right
3192882
2650083
3607701
Right
745308
99841
790646
788660
64311
109614
111
3192883
2650042
3607702
Left
3192884
2650084
3607703
Left
3192885
2650085
3607704
Left
3192886
2650086
3607705
Left
3192887
2650087
3607706
Right
3192888
2650088
3607707
Right
3192889
2650089
3607708
Right
3192890
2650090
3607709
Right
745309
99835
790647
788661
64311
109639
112
3192891
2650091
3607710
Left
3192892
2650092
3607711
Left
3192893
2650093
3607712
Left
3192894
2650038
3607713
Right
3192895
2650094
3607714
Right
745310
99878
790648
788662
64311
2627
111
3192896
2650095
3607715
Left
3192897
2650096
3607716
Left
3192898
2650038
3607717
Right
3192899
2650097
3607718
Right
745311
2616
790649
788663
64311
108571
112
3192900
2650038
3607720
Left
3192901
2650099
3607721
Left
3192902
2650100
3607722
Left
3192903
2650096
3607723
Right
3192904
2650101
3607724
Right
3192905
2650102
3607725
Right
745312
98728
790650
788664
64311
109689
111
3192906
2650103
3607726
Left
3192907
2650096
3607727
Left
3192908
2650104
3607728
Right
3192909
2650038
3607729
Right
745313
99962
790651
788665
64311
109690
111
3192910
2650096
3607731
Left
3192911
2650106
3607732
Left
3192912
2650107
3607733
Right
3192913
2650108
3607734
Right
1391
3414
3607735
Left
1392
3415
3607736
Right
745314
99963
790652
788666
64311
109691
111
3192914
2650038
3607737
Left
3192915
2650109
3607738
Left
3192916
2650110
3607739
Left
3192917
2650111
3607740
Right
3192918
2650112
3607741
Right
3192919
2650113
3607742
Right
745315
99964
790653
788667
64311
109641
111
3192920
2650111
3607743
Left
3192921
2650114
3607744
Left
3192922
2650115
3607745
Left
3192923
2650116
3607746
Right
3192924
2650117
3607747
Right
3192925
2650118
3607748
Right
745316
99881
790654
788668
64311
109692
112
3192926
2650120
3607750
Left
3192927
2650121
3607751
Left
3192928
2650116
3607752
Right
3192929
2650122
3607753
Right
745317
99965
790655
788669
64311
109647
111
3192930
2650038
3607755
Left
3192931
2650124
3607756
Right
3192932
2650125
3607757
Right
745318
99893
790656
788670
64311
109649
111
3192933
2650124
3607759
Left
3192934
2650127
3607760
Left
3192935
2650128
3607761
Right
3192936
2650129
3607762
Right
745319
99897
790657
788671
64311
109693
112
3192937
2650128
3607764
Left
3192938
2650131
3607765
Left
3192939
2650132
3607766
Left
3192940
2650133
3607767
Right
3192941
2650134
3607768
Right
3192942
2650135
3607769
Right
745320
99966
790658
788672
64311
108868
3192943
2650042
3607770
Left
3192944
2650136
3607771
Left
3192945
2650137
3607772
Right
3192946
2650138
3607773
Right
1393
3416
3607774
Left
1394
3417
3607775
Right
745321
99051
790659
107486
107904
64311
14
false
2302
1505
16
regular
104254
2650038
3607776
Right
107487
107906
64311
14
false
479
1632
16
regular
104255
2650062
3607777
Left
107488
107907
64311
14
false
244
1103
16
regular
104256
2650070
3607778
Left
107489
107903
64311
14
false
2297
1610
16
regular
104257
2650038
3607779
Right
107490
107905
64311
14
false
2302
1365
16
regular
104258
2650038
3607780
Left
107491
107908
64311
14
false
1245
2690
16
regular
104259
2650124
3607781
Left
107492
107902
64311
14
false
1067
2145
16
regular
104260
2650091
3607782
Right
184563
13
1740
1195
0.6
0.6
0
2
1
490
240
184564
13
300
1370
0.6
0.6
0
2
1
490
240
184565
13
1270
1915
0.6
0.6
5
2
1
490
240
184566
13
1725
1745
0.9
0.9
0
2
1
490
240
184567
13
1095
2270
0.6
0.6
0
2
1
490
240
184568
13
685
2425
1.3
1.3
90
2
1
490
240
184569
13
1165
3040
0.6
0.6
0
2
1
490
240
368036
M66 140 C66 90 116 40 166 40 C768 40 1551 40 2153 40 C2203 40 2253 90 2253 140 C2253 1052 2253 2236 2253 3148 C2253 3198 2203 3248 2153 3248 C1551 3248 768 3248 166 3248 C116 3248 66 3198 66 3148 C66 2236 66 1052 66 140
1
true
6
2187.0
3208.0
446199
15
Intracellular Space
130
105
20
1.0
1.0
160
15
2-Hydroxyglutric Aciduria (D and L Form)
L-2-Hydroxyglutaric Aciduria (D-2-Hydroxyglutaric Aciduria ) is an autosomal recessive disease caused by a mutation in the L2HGDH gene which codes for L-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of L-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; and L-lysine in plasma and spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results. D-2-Hydroxyglutaric Aciduria is an autosomal recessive disease caused by a mutation in the D2HGDH gene which does for D-2-Hydroxygluarate dehydrogenase. A deficiency in this enzyme results in accumulation of D-2-Hydroxyglutaric acid in plasma, spinal fluid, and urine; oxoglutaric acid in urine; and gabba-aminobutyric acid in spinal fluid. Symptoms, which present at birth, include ataxia, hypotonia, mental retardation, and seizures. Premature death often results.
Disease
PW_X008896
Complete
Context8896
48262
7736
Protein
Mutated
48263
546
Compound
Increased
48264
8
Tissue
Damaged
48265
14
Tissue
Damaged
48266
24
Tissue
Damaged
48267
15
Tissue
Damaged
48268
11
Tissue
Damaged
278471
16601864
Struys EA: D-2-Hydroxyglutaric aciduria: unravelling the biochemical pathway and the genetic defect. J Inherit Metab Dis. 2006 Feb;29(1):21-9. doi: 10.1007/s10545-006-0317-9.
8896
Context
278472
22391998
Kranendijk M, Struys EA, Salomons GS, Van der Knaap MS, Jakobs C: Progress in understanding 2-hydroxyglutaric acidurias. J Inherit Metab Dis. 2012 Jul;35(4):571-87. doi: 10.1007/s10545-012-9462-5. Epub 2012 Mar 6.
8896
Context
279307
25763823
Rzem R, Achouri Y, Marbaix E, Schakman O, Wiame E, Marie S, Gailly P, Vincent MF, Veiga-da-Cunha M, Van Schaftingen E: A mouse model of L-2-hydroxyglutaric aciduria, a disorder of metabolite repair. PLoS One. 2015 Mar 12;10(3):e0119540. doi: 10.1371/journal.pone.0119540. eCollection 2015.
8896
Context