2264PathwayLeucine DegradationThe degradation of L-leucine starts either in the mitochondria or the cytosol. L-leucine reacts with 2-oxoglutarate through a branch-chain amino acid aminotransferase resulting in the release of ketoleucine and glutamate. The latter compound reacts with ketoisocaproate decarboxylase resulting in the release of carbon dioxide and 3-methylbutanal. The latter compound can then be turned into 3-methylbutanol through a alcohol dehydrogenaseMetabolicPW002490CenterPathwayVisualizationContext277619522474#000099PathwayVisualization22472264Leucine DegradationThe degradation of L-leucine starts either in the mitochondria or the cytosol. L-leucine reacts with 2-oxoglutarate through a branch-chain amino acid aminotransferase resulting in the release of ketoleucine and glutamate. The latter compound reacts with ketoisocaproate decarboxylase resulting in the release of carbon dioxide and 3-methylbutanal. The latter compound can then be turned into 3-methylbutanol through a alcohol dehydrogenaseMetabolic18527810989420Dickinson JR: Pathways of leucine and valine catabolism in yeast. Methods Enzymol. 2000;324:80-92.2264Pathway527912499363Dickinson JR, Salgado LE, Hewlins MJ: The catabolism of amino acids to long chain and complex alcohols in Saccharomyces cerevisiae. J Biol Chem. 2003 Mar 7;278(10):8028-34. doi: 10.1074/jbc.M211914200. Epub 2002 Dec 23.2264Pathway52809748245Dickinson JR, Harrison SJ, Hewlins MJ: An investigation of the metabolism of valine to isobutyl alcohol in Saccharomyces cerevisiae. J Biol Chem. 1998 Oct 2;273(40):25751-6.2264Pathway52818702755Eden A, Simchen G, Benvenisty N: Two yeast homologs of ECA39, a target for c-Myc regulation, code for cytosolic and mitochondrial branched-chain amino acid aminotransferases. J Biol Chem. 1996 Aug 23;271(34):20242-5.2264Pathway52828798704Kispal G, Steiner H, Court DA, Rolinski B, Lill R: Mitochondrial and cytosolic branched-chain amino acid transaminases from yeast, homologs of the myc oncogene-regulated Eca39 protein. J Biol Chem. 1996 Oct 4;271(40):24458-64.2264Pathway1CellCL:00000006MyocyteCL:00001875HepatocyteCL:00001823NeuronCL:00005401Homo sapiens9606EukaryoteHuman3Escherichia coli562Prokaryote18Saccharomyces cerevisiae4932EukaryoteYeast23Pseudomonas aeruginosa287Prokaryote12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat24Solanum lycopersicum4081EukaryoteTomato21Xenopus laevis8355EukaryoteAfrican clawed frog4Arabidopsis thaliana3702EukaryoteThale cress49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia410Drosophila melanogaster7227EukaryoteFruit fly6Caenorhabditis elegans6239EukaryoteRoundworm2Bacteria2ProkaryoteBacteria25Escherichia coli (strain K12)83333Prokaryote19Schizosaccharomyces pombe4896Eukaryote7Chlamydomonas reinhardtii3055Eukaryote29Saccharomyces cerevisiae (strain ATCC 204508 / S288c)559292EukaryoteBaker's yeast1CytosolGO:000582911Extracellular SpaceGO:000561531Periplasmic SpaceGO:00056202MitochondrionGO:00057393Mitochondrial MatrixGO:00057595CytoplasmGO:000573713Endoplasmic ReticulumGO:000578324Mitochondrial Intermembrane SpaceGO:00057584PeroxisomeGO:000577710Cell MembraneGO:000588635ChloroplastGO:00095076LysosomeGO:00057647Endoplasmic Reticulum MembraneGO:000578912Mitochondrial Inner MembraneGO:000574314Mitochondrial Outer MembraneGO:000574136MembraneGO:001602053Endoplasmic Reticulum BodyGO:001016834Plant-Type VacuoleGO:000032532Inner MembraneGO:007025825Golgi apparatusGO:000579416Lysosomal LumenGO:00432021LiverBTO:00007597299MuscleBTO:00008871411824BrainBTO:0000142891628StomachBTO:0001307155268Blood VesselBTO:000110274114Adrenal MedullaBTO:00000497182111PW_BS00000215111PW_BS000015107313PW_BS00010710813PW_BS00010816611PW_BS000166188118PW_BS0000241873118PW_BS000024315123PW_BS0000243183123PW_BS0000241321121PW_BS000132124151PW_BS0001241181171PW_BS0001183211PW_BS0000034311PW_BS0000048511PW_BS000008181311PW_BS000018311511PW_BS000031422411PW_BS0000425411PW_BS000005509516PW_BS000050261115PW_BS000026541315PW_BS000054103331PW_BS000103117131PW_BS0001171471241PW_BS0001471553241PW_BS0001551572241PW_BS0001571613181PW_BS0001611783211PW_BS00017885241011PW_BS000085222341PW_BS000024224241PW_BS000024151141PW_BS0001512253541PW_BS00002422014PW_BS0000241985181PW_BS0000242892491PW_BS000024253541PW_BS0000241333121PW_BS0001331115121PW_BS0001111122121PW_BS00011212915121PW_BS00012934524121PW_BS00002834695126PW_BS00002832711125PW_BS00002834713125PW_BS0000283344121PW_BS0000283683601PW_BS0000281355171PW_BS000135943PW_BS000094406351PW_BS000115407251PW_BS000115122551PW_BS0001224141551PW_BS0001154182451PW_BS000115408451PW_BS0001154239556PW_BS0001154241155PW_BS0001154251355PW_BS0001151203171PW_BS0001201192171PW_BS00011945015171PW_BS00011545424171PW_BS0001153744171PW_BS00005345895176PW_BS00011545911175PW_BS00011546013175PW_BS0001154793101PW_BS0001152991101PW_BS0000244812101PW_BS0001152975101PW_BS00002448924101PW_BS0001154824101PW_BS000115501361PW_BS000115388161PW_BS000112206261PW_BS000024205561PW_BS0000245062461PW_BS000115502461PW_BS00011511PW_BS00000114101PW_BS0000149611PW_BS0000091136121PW_BS000113105113PW_BS00010572513PW_BS000072711113PW_BS000071207661PW_BS0000242905491PW_BS0000243201123PW_BS000024126651PW_BS0001264436171PW_BS0001153016101PW_BS00002430412PW_BS00002449711PW_BS000049171211PW_BS000017221411PW_BS000022101711PW_BS0000107028511PW_BS000070100521PW_BS00010015924PW_BS00015915284PW_BS000152101531PW_BS0001011632181PW_BS000163219314PW_BS0000242137181PW_BS00002421013181PW_BS00002421217181PW_BS0000241601181PW_BS00016017018PW_BS000170226441PW_BS00002416212181PW_BS0001621951318PW_BS0000242491341PW_BS0000241644PW_BS0001642811251PW_BS0000242851041PW_BS0000242863641PW_BS0000242875341PW_BS0000242273441PW_BS0000242231241PW_BS0000242941141PW_BS0000243081011PW_BS0000243221231PW_BS0000243125231PW_BS00002429341PW_BS00002413412121PW_BS0001343317121PW_BS0000281141112PW_BS00011413013121PW_BS000130310312PW_BS000024383751PW_BS000100390761PW_BS0001123987171PW_BS0001133361121PW_BS000028109323PW_BS000109409115PW_BS0001153841251PW_BS0001001251351PW_BS0001251371117PW_BS00013712112171PW_BS00012113613171PW_BS0001364831110PW_BS0001154957101PW_BS00011548012101PW_BS00011530013101PW_BS000024208116PW_BS0000243911261PW_BS0001123951361PW_BS00011313121PW_BS000013204111PW_BS000020432511PW_BS0000431901118PW_BS0000242771218PW_BS0000243331212PW_BS00002834141121PW_BS00002835625121PW_BS000028412125PW_BS0001154192551PW_BS0001154461217PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS00011529111PW_BS0000292811611PW_BS0000286131PW_BS000006951721PW_BS0000951231751PW_BS00012314117191PW_BS0001412164181PW_BS00002429817101PW_BS00002433217121PW_BS000028429151PW_BS00011544717171PW_BS0001154641171PW_BS000115644171PW_BS0005087081291PW_BS000512540L-LeucineHMDB0000687Branched chain amino acids (BCAA) are essential amino acids whose carbon structure is marked by a branch point. These three amino acids are critical to human life and are particularly involved in stress, energy and muscle metabolism. BCAA supplementation as therapy, both oral and intravenous, in human health and disease holds great promise. 'BCAA' denotes valine, isoleucine and leucine which are branched chain essential amino acids. Despite their structural similarities, the branched amino acids have different metabolic routes, with valine going solely to carbohydrates, leucine solely to fats and isoleucine to both. The different metabolism accounts for different requirements for these essential amino acids in humans: 12 mg/kg, 14 mg/kg and 16 mg/kg of valine, leucine and isoleucine respectively. Furthermore, these amino acids have different deficiency symptoms. Valine deficiency is marked by neurological defects in the brain, while isoleucine deficiency is marked by muscle tremors. Many types of inborn errors of BCAA metabolism exist, and are marked by various abnormalities. The most common form is the maple syrup urine disease, marked by a characteristic urinary odor. Other abnormalities are associated with a wide range of symptoms, such as mental retardation, ataxia, hypoglycemia, spinal muscle atrophy, rash, vomiting and excessive muscle movement. Most forms of BCAA metabolism errors are corrected by dietary restriction of BCAA and at least one form is correctable by supplementation with 10 mg of biotin daily. BCAA are useful because they are metabolized primarily by muscle. Stress state- e.g surgery, trauma, cirrhosis, infections, fever and starvation--require proportionately more BCAA than other amino acids and probably proportionately more leucine than either valine or isoleucine. BCAA and other amino acids are frequently fed intravenously (TPN) to malnourished surgical patients and in some cases of severe trauma. BCAA, particularly leucine, stimulate protein synthesis, increase reutilization of amino acids in many organs and reduce protein breakdown. Furthermore, leucine can be an important source of calories, and is superior as fuel to the ubiquitous intravenous glucose (dextrose). Leucine also stimulates insulin release, which in turn stimulates protein synthesis and inhibits protein breakdown. These effects are particularly useful in athletic training. BCAA should also replace the use of steroids as commonly used by weightlifters. Huntington's chorea and anorexic disorders both are characterized by low serum BCAA. These diseases, as well as forms of Parkinson's, may respond to BCAA therapy. BCAA, and particularly leucine, are among the amino acids most essential for muscle health. (http://www.dcnutrition.com).61-90-5C00123610615603LEU5880DB00149CC(C)C[C@H](N)C(O)=OC6H13NO2InChI=1S/C6H13NO2/c1-4(2)3-5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t5-/m0/s1ROHFNLRQFUQHCH-YFKPBYRVSA-N(2S)-2-amino-4-methylpentanoic acid131.1729131.094628665-0.272L-leucine00FDB001946(2s)-2-amino-4-methylpentanoate;(2s)-2-amino-4-methylpentanoic acid;(s)-(+)-leucine;(s)-2-amino-4-methylpentanoate;(s)-2-amino-4-methylpentanoic acid;(s)-2-amino-4-methylvalerate;(s)-2-amino-4-methylvaleric acid;(s)-leucine;4-methyl-l-norvaline;L-(+)-leucine;L-a-aminoisocaproate;L-a-aminoisocaproic acid;L-alpha-aminoisocaproate;L-alpha-aminoisocaproic acid;Leu;Leucine;(2s)-alpha-2-amino-4-methylvaleric acid;(2s)-alpha-leucine;2-amino-4-methylvaleric acid;L;L-leucin;L-leuzin;(2s)-a-2-amino-4-methylvalerate;(2s)-a-2-amino-4-methylvaleric acid;(2s)-alpha-2-amino-4-methylvalerate;(2s)-α-2-amino-4-methylvalerate;(2s)-α-2-amino-4-methylvaleric acid;(2s)-a-leucine;(2s)-α-leucinePW_C000540Leu1582250431556461075647108684816671451887146187425393154255731879181132121544124124102118134Oxoglutaric acidHMDB0000208Oxoglutaric 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).328-50-7C0002651309152-KETOGLUTARATE50DB02926OC(=O)CCC(=O)C(O)=OC5H6O5InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)KPGXRSRHYNQIFN-UHFFFAOYSA-N2-oxopentanedioic acid146.0981146.021523302-0.442oxoglutarate0-2FDB0033612-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 acidPW_C000134AKG152423141414684991867331110842126351447501455261467545375103541411754381185564132600814760361556069157609216164821786530857471222751522475191518209225837422011863198126812897705425377135133774811117752311277746129779673457797034677976327779843477842533480018368806941351131629411997240612002212412008440712017412212055241412081441812098940812114642312115242412116042512275712012283111912318645012339945412355437412371845812372445912373246012535747912540029912545548112553329712580048912592948212690050112694038812699320612706620512725550612738850295L-Glutamic acidHMDB0000148Glutamic 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).56-86-0C000253303216015GLT30572DB00142N[C@@H](CCC(O)=O)C(O)=OC5H9NO4InChI=1S/C5H9NO4/c6-3(5(9)10)1-2-4(7)8/h3H,1-2,6H2,(H,7,8)(H,9,10)/t3-/m0/s1WHUUTDBJXJRKMK-VKHMYHEASA-N(2S)-2-aminopentanedioic acid147.1293147.053157781-0.263L-glutamic acid0-1FDB012535(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-glutaminsaeurePW_C000095Glu16244365811911384164149699110542144850145626146254532311153441135415117543911855651325631107563210858591056006147607115761919465318568381876844188709272709371716520571822077514224751815182082258373220117921981185516112004222126213112683289126972904234831542349318428453207702025377332133775251127797134677977327779813477829134580649135120023124120040122120086407120347406120692126120816418121147423121153424121157425122833119122997120123299443123401454123719458123725459123729460125401299125418297125457481125667479125769301125802489126941388126995206127162501127257506547KetoleucineHMDB0000695Ketoleucine is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. Ketoleucine is both 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 ketoleucine are associated with maple syrup urine disease (MSUD). MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465).816-66-0C0023370484302K-4CH3-PENTANOATE69DB03229CC(C)CC(=O)C(O)=OC6H10O3InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)BKAJNAXTPSGJCU-UHFFFAOYSA-N4-methyl-2-oxopentanoic acid130.1418130.062994186-1.281ketoisocaproate0-1FDB0126072-keto-4-methylvalerate;2-keto-4-methylvaleric acid;2-ketoisocaproate;2-ketoisocaproic acid;2-oxo-4-methylpentanoate;2-oxo-4-methylpentanoic acid;2-oxo-4-methylvalerate;2-oxo-4-methylvaleric acid;2-oxoisocaproate;2-oxoisocaproic acid;2-oxoleucine;4-methyl-2-oxo-valerate;4-methyl-2-oxo-valeric acid;4-methyl-2-oxopentanoate;4-methyl-2-oxopentanoic acid;Ketoisocaproate;Ketoisocaproic acid;Methyloxovalerate;Methyloxovaleric acid;Oxoisocaproate;Oxoisocaproic acid;A-ketoisocaproate;A-ketoisocaproic acid;A-ketoisocapronate;A-ketoisocapronic acid;A-oxoisocaproate;A-oxoisocaproic acid;Alpha-keto-isocaproate;Alpha-keto-isocaproic acid;Alpha-ketoisocaproate;Alpha-ketoisocaproic acid;Alpha-ketoisocapronate;Alpha-ketoisocapronic acid;Alpha-oxoisocaproate;Alpha-oxoisocaproic acid;Keto-leucinePW_C000547Ketoleu1583273741887524220132323047918213212154512412410311840034Hydrogen IonHMDB0059597Hydrogen 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])C000801038153781010[H+]HInChI=1S/p+1GPRLSGONYQIRFK-UHFFFAOYSA-Nhydron1.00791.0078250320hydron10H+;H(+);Hydrogen cation;Hydron;ProtonPW_C040034H+2154670875315788318483111621463261464542231492780174250224254424547104576184694705241103532711153531125626108563910756991005720105574211759631476037155607015760931616130159623216664831786601152669210168431886910187710016371682057191206745321974542207472222752521375322107558212757216075901708195225821815182432268413162842022491391959155249119151641201528112181285122462861226628712521227132572231332529415330308423293154235431842401322424053124245432076912293771361337721013477372331778041147795513277990327779913477837934579929130800193688038731080388304807221199382312494823383110550388112855941132803901155373981155391181158563361162051091199734061201934071205491221205934091211704241211714251225694181226153841226871251227581201231831351232181371237424591237434601251414541251881211252731361253594791255504811257304831257362971258092991265174951267174891267664801268233001269025011272132081283085061283613911284303952763Iso-ValeraldehydeHMDB0006478iso-Valeraldehyde is an oxygenated (aldehyde) volatile organic compounds (VOC), normally found in humans, particularly in the gut. iso-Valeraldehyde is a leucine degradation product that has been found in elevated concentrations in some cases of hepatic encephalopathy, however not significantly. Fermentation of carbohydrates in the gut produces ethanoic, propionic, butanoic, pentanoic, and hexanoic acid acids, particularly by Bacteroides, although it is possible that some of the iso-Valeraldehyde is of dietetic origin. iso-Valeraldehyde has been found to be a normal component of the chemical profile of cheddar cheese, spirits (beer, rum, sake, wine). (PMID: 17314143, 18275147, 18175918, 16899660).590-86-3C07329115521663811065CC(C)CC=OC5H10OInChI=1S/C5H10O/c1-5(2)3-4-6/h4-5H,3H2,1-2H3YGHRJJRRZDOVPD-UHFFFAOYSA-N3-methylbutanal86.132386.073164942-0.710isovaleraldehyde00FDB0032852-methylbutanal-4;3-methyl-1-butanal;3-methyl-butanal;3-methyl-butyraldehyde;3-methylbutan-1-al;3-methylbutanal;3-methylbutyraldehyde;Iso-c4h9cho;Isoamylaldehyde;Isopentaldehyde;Isopentanal;Isovaleral;Isovaleraldehyde;Isovaleric aldehyde;Isovalerylaldehyde;Methyl butanal;B-methylbutanal;Beta-methylbutanal;Isoamyl aldehyde;β-methylbutanalPW_C002763IsoVal1316Carbon dioxideHMDB0001967Carbon 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.124-38-9C0001128016526274O=C=OCO2InChI=1S/CO2/c2-1-3CURLTUGMZLYLDI-UHFFFAOYSA-Nmethanedione44.009543.9898292440.630carbon dioxide00DBMET00423FDB014084Carbon oxide;Carbon-12 dioxide;Carbonic acid anhydride;Carbonic acid gas;Carbonic anhydride;[co2];Co2;E 290;E-290;E290;R-744PW_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021144NADHHMDB0001487NADH 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).58-68-4C0000443915316908NADH388299DB00157NC(=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]1OC21H29N7O14P2InChI=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/s1BOPGDPNILDQYTO-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 acid665.441665.124771695-2.358NADH0-2FDB0226491,4-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 dinucleotidePW_C001144NADH14341533490864810111521275514695422304927811728362931099480618481218482128490464959315169955240103533211153581125466123547912555931355698100573710858291415915147594515160271556079161638716472178677111768931607011188709916371722057195206746222282442268360225908622411809198118212161232024913003298130153001325522342403322426183157710713277123133772081347737133177651336776683347770033277707130779171137798634780009368806911199382212411054938811285494115838118119955406120172407120378122120986408121162425121244126121693429121818383122616384122745120123127447123138136123551374123734460123814443124242464124371398125189121125345479125531481125762297125808299125926482126516495126767480126888501127385502128090390128362391128429395721NADHMDB0000902NAD (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).53-84-9C00003589315846NAD5682NC(=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]1OC21H28N7O14P2InChI=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/s1BAWFJGJZGIEFAR-NNYOXOHSSA-O1-[(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-ylium664.433664.116946663-2.5981-[(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-ylium1-1FDB0223093-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-nadPW_C000721NAD140415033538651101114211344312735146654222949277917283529310794807184813184819284902649603151679552381035334111536011254691235482125559013556101185696100573810858271415912147594215160241556072157607616163851646917867721176890160701218870971637174205719720674051987459222824122683592259085224118192161232224913006298130183001325622342404322426193157710413277120133772091347737033177650336776673347770233277709130779151137798334778406356800063688069011993825124110552388112750166112853941199291221199524061201714071208344191209844081211594251212421261212594291218173831226143841227421201231304471231411361234194551235493741237314601238124431238294641243703981251871211253192971253424791255304811258062991258254901259244821265154951267654801268855011272785071273835021280893901283603911284283952619IsopentanolHMDB0006007Isopentanol or Isoamyl alcohol is one of several isomers of amyl alcohol. It is a by-product of gut microbial fermentation (PMID: 17452087). It can be produced by 3-methylbutanal reductase (EC 1.1.1.265) from 3 methylbutanal. Isoamyl alcohol is the major higher chain alcohol in alcoholic beverages and is present in cider, mead, beer, wine, and spirits to varying degrees, being obtained by the fermentation of starches. Isopentanol has been shown to induce expression of CYP3A and CYP2E1 in human liver (PMID: 7574728).123-51-3C073283126015837CPD-703229000DB02296CC(C)CCOC5H12OInChI=1S/C5H12O/c1-5(2)3-4-6/h5-6H,3-4H2,1-2H3PHTQWCKDNZKARW-UHFFFAOYSA-N3-methylbutan-1-ol88.148288.088815006-0.371isoamyl alcohol00FDB0081311-hydroxy-3-methylbutane;2-methyl-4-butanol;3-methyl-1-butanol;3-methyl-butanol;3-methyl-butan-(1)-ol;3-methylbutan-1-ol;3-methylbutanoi;3-methylbutanol;Butan-1-ol, 3-methyl;Fermentation amyl alcohol;Fusel oil;I-amyl alcohol;Iso-amyl alcohol;Iso-amylalkohol;Isoamyl alcohol;Isoamyl alcohol (3-methyl butanol);Isoamyl alcohol (natural);Isoamyl alkohol;Isoamylalcohol;Isoamylol;Isobutyl carbinol;Isobutylcarbinol;Isopentan-1-ol;Isopentanol;Isopentyl alcohol;Methyl-3-butan-1-ol;Primary isoamyl alcoholPW_C002619Isopent10936Branched-chain-amino-acid aminotransferase, mitochondrialP38891Involved in the biosynthesis of the branched chain amino acids leucine, isoleucine, and valine. Catalyzes the formation of methionine from 2-keto-4-methylthiobutyrate (KMTB) in the methionine salvage pathway primarily using branched chain amino acids (leucine, isoleucine, and valine) as the amino donors. Appears to be involved in the regulation of the transition from G1 to S phase in the cell cycle.BAT1292.6.1.42784016310937Branched-chain-amino-acid aminotransferase, cytosolicP47176
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. Catalyzes the formation of methionine from 2-keto-4-methylthiobutyrate (KMTB) in the methionine salvage pathway primarily using branched chain amino acids (leucine, isoleucine, and valine) as well as lysine and proline as the amino donors. Involved in cell cycle regulation.
BAT2292.6.1.42784116010938ketoisocaproate decarboxylase / decarboxylaseQ07471
One of five 2-oxo acid decarboxylases (PDC1, PDC5, PDC6, ARO10, and THI3) involved in amino acid catabolism. The enzyme catalyzes the decarboxylation of amino acids, which, in a first step, have been transaminated to the corresponding 2-oxo acids (alpha-keto-acids). In a third step, the resulting aldehydes are reduced to alcohols, collectively referred to as fusel oils or alcohols. Its preferred substrates are the transaminated amino acids leucine and isoleucine, whereas valine, aromatic amino acids, and pyruvate are no substrates. In analogy to the pyruvate decarboxylases the enzyme may in a side-reaction catalyze condensation (or carboligation) reactions leading to the formation of 2-hydroxy ketone, collectively called acyloins. The enzyme is also positively regulating the thiamine metabolism by a molecular mechanism that may involve thiamine concentration sensing and signal transmission.
THI3, KID1294.1.1.-784916010939Decarboxylase Q06408
One of five 2-oxo acid decarboxylases (PDC1, PDC5, PDC6, ARO10, and THI3) involved in amino acid catabolism. The enzyme catalyzes the decarboxylation of amino acids, which, in a first step, have been transaminated to the corresponding 2-oxo acids (alpha-keto-acids). In a third step, the resulting aldehydes are reduced to alcohols, collectively referred to as fusel oils or alcohols. Its preferred substrates are the transaminated amino acids, phenylalanine, tryptophan, (and probably tyrosine), but also isoleucine, whereas leucine is a low efficiency and valine and pyruvate are no substrates. In analogy to the pyruvate decarboxylases the enzyme may in a side-reaction catalyze condensation (or carboligation) reactions leading to the formation of 2-hydroxy ketone, collectively called acyloins.
ARO10294.1.1.43784816010641alcohol dehydrogenase subunit IP25377
NADP-dependent alcohol dehydrogenase with a broad substrate specificity.
ADH7291.1.1.2775516013609364413708870810642alcohol dehydrogenase subunit 2Q04894
NADP-dependent alcohol dehydrogenase with a broad substrate specificity.
ADH6291.1.1.277561601360946441370897084713Branched-chain-amino-acid aminotransferase, mitochondrial18PW_P00471311894109364714Branched-chain-amino-acid aminotransferase, cytosolic18PW_P00471411895109374715ketoisocaproate decarboxylase / decarboxylase18PW_P004715118961093811897109394337alcohol dehydrogenase18PW_P004337113981064111399106426973falsePW_R006973Right287195401Compoundfalse287201341Compoundtrue28721951Compoundtrue287225471Compoundfalse695047136974falsePW_R006974Right287235401Compoundfalse287241341Compoundtrue28725951Compoundtrue287265471Compoundfalse695147146975falsePW_R006975Right287275471Compoundfalse28728400341Compoundfalse2872927631Compoundfalse2873013161Compoundfalse695247154.1.1.-6976falsePW_R006976Both2873127631Compoundfalse2873211441Compoundfalse28733400341Compoundfalse287347211Compoundfalse2873526191Compoundfalse69534337560PW_T0005607065471Compound162188Right813005403false39549810regular100100813011343false50060310regular10011081302953false89559810regular100110813035473false104049310regular100100813045471883false104089210regular100100813055403false39589310regular100100813061343false49599310regular10011081307953false93098810regular100110813084003455false1201100410regular78788130927633false173089110regular10011081310131652false1596100610regular787881311114460false1952112610regular5030813124003455false1942100210regular78788131372159false1611142610regular50308131426193false1731148610regular10010038645109361632false6705108subunitregular1507038646109371602false6859058subunitregular1507038647109381602false12858738subunitregular1507038648109391602false13609088subunitregular1507038649106411602false162312088subunitregular1507038650106421602false170312488subunitregular150703145747132247379693864531458471422473797038646314594715224737971386473797238648314604337224737973386493797438650116716M495 547 C525 547 640 545 670 545 5false18116717M600 658 C593 549 640 545 670 545 5false18116718M895 653 C887 549 850 545 820 545 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false116719M1040 543 C1010 543 850 545 820 545 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false116720M1090 593 C1090 623 1090 638 1090 668 83false18116721M1090 892 C1090 862 1090 698 1090 668 83false18trueM 639.9468550441649 450.261556296296 L 625 449 L 631.3808877211858 462.5751343230783false116722M495 943 C525 943 655 940 685 940 5false18116723M595 1048 C599 940 655 940 685 940 5false18116724M930 1043 C929 946 865 940 835 940 5false18trueM 639.9468550441649 582.261556296296 L 625 581 L 631.3808877211858 594.5751343230784false116725M1040 942 C1010 942 865 940 835 940 5false18trueM 639.9468550441649 582.261556296296 L 625 581 L 631.3808877211858 594.5751343230784false116726M1140 942 C1170 942 1330 943 1360 943 5false18116727M1279 1043 C1283 950 1330 943 1360 943 5false18116728M1730 946 C1700 946 1540 943 1510 943 5false18trueM 664.9468550441649 711.261556296296 L 650 710 L 656.3808877211858 723.5751343230784false116729M1596 1045 C1591 945 1540 943 1510 943 5false18trueM 664.9468550441649 711.261556296296 L 650 710 L 656.3808877211858 723.5751343230784false116730M1780 1001 C1780 1031 1778 1218 1778 1248 5false18trueM 1426.9468550441647 996.261556296296 L 1412 995 L 1418.380887721186 1008.5751343230784false116731M1977 1156 C1776 1162 1775 1187 1778 1248 5false18trueM 1426.9468550441647 996.261556296296 L 1412 995 L 1418.380887721186 1008.5751343230784false116732M1981 1080 C1774 1080 1777 1116 1778 1248 5false18trueM 1426.9468550441647 996.261556296296 L 1412 995 L 1418.380887721186 1008.5751343230784false116733M1636 1426 C1729 1425 1778 1348 1778 1318 5false18trueM 1426.9468550441647 996.261556296296 L 1412 995 L 1418.380887721186 1008.5751343230784false116734M1781 1486 C1781 1456 1778 1348 1778 1318 5false18trueM 1426.9468550441647 996.261556296296 L 1412 995 L 1418.380887721186 1008.5751343230784false23951224769739233281300116716Left9233381301116717Left9233481302116718Right9233581303116719Right2375069503145723952224769749233681305116722Left9233781306116723Left9233881307116724Right9233981304116725Right2375169513145823953224769759234081304116726Left9234181308116727Left9234281309116728Right9234381310116729Right2375269523145923954224769769234481309116730Left9234581311116731Left9234681312116732Left9234781313116733Right9234881314116734Right2375369533146010625602247257981303116720Left258081304116721Right154118903781.01.0902904844974378M125 225 C125 175 175 125 225 125 C810 125 1571 125 2156 125 C2206 125 2256 175 2256 225 C2256 680 2256 1272 2256 1727 C2256 1777 2206 1827 2156 1827 C1571 1827 810 1827 225 1827 C175 1827 125 1777 125 1727 C125 1272 125 680 125 225 1true62131.01702.04379M274 374 C274 324 324 274 374 274 C869 274 1513 274 2008 274 C2058 274 2108 324 2108 374 C2108 741 2108 1218 2108 1585 C2108 1635 2058 1685 2008 1685 C1513 1685 869 1685 374 1685 C324 1685 274 1635 274 1585 C274 1218 274 741 274 374 1true61834.01411.07321421591354360118974940#FFEBEB4835389