88222PathwayVitamin K MetabolismVitamin K describes a group of lipophilic, hydrophobic vitamins that exist naturally in two forms (and synthetically in three others): vitamin K1, which is found in plants, and vitamin K2, which is synthesized by bacteria. Vitamin K is an important dietary component because it is necessary as a cofacter in the activation of vitamin K dependent proteins. Metabolism of vitamin K occurs mainly in the liver. In the first step, vitamin K is reduced to its quinone form by a quinone reductase such as NAD(P)H dehydrogenase. Reduced vitamin K is the form required to convert vitamin K dependent protein precursors to their active states. It acts as a cofactor to the integral membrane enzyme vitamin K-dependent gamma-carboxylase (along with water and carbon dioxide as co-substrates), which carboxylates glutamyl residues to gamma-carboxy-glutamic acid residues on certain proteins, activating them. Each converted glutamyl residue produces a molecule of vitamin K epoxide, and certain proteins may have more than one residue requiring carboxylation. To complete the cycle, the vitamin K epoxide is returned to vitamin K via the vitamin K epoxide reductase enzyme, also an integral membrane protein. The vitamin K dependent proteins include a number of important coagulation factors, such as prothrombin. Thus, warfarin and other coumarin drugs act as anticoagulants by blocking vitamin K epoxide reductase.MetabolicPW088340CenterPathwayVisualizationContext8861622001750#000099PathwayVisualization8809688222Vitamin K MetabolismVitamin K describes a group of lipophilic, hydrophobic vitamins that exist naturally in two forms (and synthetically in three others): vitamin K1, which is found in plants, and vitamin K2, which is synthesized by bacteria. Vitamin K is an important dietary component because it is necessary as a cofacter in the activation of vitamin K dependent proteins. Metabolism of vitamin K occurs mainly in the liver. In the first step, vitamin K is reduced to its quinone form by a quinone reductase such as NAD(P)H dehydrogenase. Reduced vitamin K is the form required to convert vitamin K dependent protein precursors to their active states. It acts as a cofactor to the integral membrane enzyme vitamin K-dependent gamma-carboxylase (along with water and carbon dioxide as co-substrates), which carboxylates glutamyl residues to gamma-carboxy-glutamic acid residues on certain proteins, activating them. Each converted glutamyl residue produces a molecule of vitamin K epoxide, and certain proteins may have more than one residue requiring carboxylation. To complete the cycle, the vitamin K epoxide is returned to vitamin K via the vitamin K epoxide reductase enzyme, also an integral membrane protein. The vitamin K dependent proteins include a number of important coagulation factors, such as prothrombin. Thus, warfarin and other coumarin drugs act as anticoagulants by blocking vitamin K epoxide reductase.Metabolic171CellCL:00000004Cardiomyocyte CL:00007465HepatocyteCL:00001822Platelet CL:00002333NeuronCL:00005407Epithelial CellCL:00000661Homo sapiens9606EukaryoteHuman12Mus musculus10090EukaryoteMouse5Bos taurus9913EukaryoteCattle17Rattus norvegicus10116EukaryoteRat3Escherichia coli562Prokaryote24Solanum lycopersicum4081EukaryoteTomato18Saccharomyces cerevisiae4932EukaryoteYeast6Caenorhabditis elegans6239EukaryoteRoundworm4Arabidopsis thaliana3702EukaryoteThale cress23Pseudomonas aeruginosa287Prokaryote10Drosophila melanogaster7227EukaryoteFruit fly21Xenopus laevis8355EukaryoteAfrican clawed frog49Bathymodiolus platifrons220390EukaryoteDeep sea mussel60Nitzschia sp.0001EukaryoteNitzschia42Bacteria2ProkaryoteBacteria19Schizosaccharomyces pombe4896Eukaryote25Escherichia coli (strain K12)83333Prokaryote5CytoplasmGO:00057372MitochondrionGO:00057397Endoplasmic Reticulum MembraneGO:00057894PeroxisomeGO:000577713Endoplasmic ReticulumGO:00057831CytosolGO:000582910Cell MembraneGO:000588619sarcoplasmic reticulumGO:001652935ChloroplastGO:00095073Mitochondrial MatrixGO:000575936MembraneGO:00160206LysosomeGO:000576412Mitochondrial Inner MembraneGO:000574316Lysosomal LumenGO:004320224Mitochondrial Intermembrane SpaceGO:000575814Mitochondrial Outer MembraneGO:000574120Endoplasmic Reticulum LumenGO:000578831Periplasmic SpaceGO:000562034Plant-Type VacuoleGO:000032539Mitochondrial membraneGO:003196611Extracellular SpaceGO:000561518Melanosome MembraneGO:003316225Golgi apparatusGO:000579421SynapseGO:004520215NucleusGO:000563453Endoplasmic Reticulum BodyGO:001016840PeriplasmGO:00425971LiverBTO:00007597295cardiocyteBTO:00015392Endothelium BTO:00003934Adrenal MedullaBTO:000004971828StomachBTO:00013071552625IntestineBTO:00006487Nervous SystemBTO:00014848Blood VesselBTO:0001102741111HeartBTO:0000562731029111PW_BS0000293361121PW_BS000028429151PW_BS0001154641171PW_BS0001158511PW_BS0000083211PW_BS000003101711PW_BS0000105411PW_BS000005181311PW_BS0000182111PW_BS00000249711PW_BS000049311511PW_BS00003114101PW_BS0000141115121PW_BS00011110813PW_BS0001081471241PW_BS00014715924PW_BS0001593551914PW_BS000035117131PW_BS000117188118PW_BS0000241632181PW_BS000163205561PW_BS0000241601181PW_BS0001602137181PW_BS00002421013181PW_BS00002421217181PW_BS00002417018PW_BS0001702253541PW_BS000024151141PW_BS000151224241PW_BS0000241985181PW_BS0000242111018PW_BS000024222341PW_BS0000241644PW_BS0001642863641PW_BS000024226441PW_BS0000242491341PW_BS000024315123PW_BS0000243221231PW_BS00002429341PW_BS0000241321121PW_BS0001323317121PW_BS00002833217121PW_BS00002813013121PW_BS0001301122121PW_BS0001121151012PW_BS0001151192171PW_BS000119943PW_BS000094407251PW_BS000115405105PW_BS000115122551PW_BS0001221231751PW_BS0001231251351PW_BS000125124151PW_BS000124383751PW_BS0001003761017PW_BS0000531355171PW_BS00013544717171PW_BS00011513613171PW_BS0001361181171PW_BS0001183987171PW_BS0001134812101PW_BS0001152975101PW_BS0000242991101PW_BS0000244957101PW_BS000115206261PW_BS000024388161PW_BS000112390761PW_BS000112261115PW_BS0000263344121PW_BS000028408451PW_BS0001153744171PW_BS0000534824101PW_BS000115502461PW_BS0001159611PW_BS0000094311PW_BS000004171211PW_BS0000172811611PW_BS000028422411PW_BS0000425811411PW_BS000058221411PW_BS0000223612011PW_BS00003613121PW_BS0000136131PW_BS000006103331PW_BS0001031021231PW_BS000102126651PW_BS00012612711651PW_BS0001271553241PW_BS00015515612241PW_BS0001561613181PW_BS00016116212181PW_BS00016211PW_BS0000011783211PW_BS00017817912211PW_BS000179107313PW_BS0001072231241PW_BS0000242164181PW_BS0000242156181PW_BS0000242273441PW_BS0000242916491PW_BS0000242924491PW_BS0000243016101PW_BS000024302116101PW_BS0000243183123PW_BS0000241333121PW_BS00013313412121PW_BS0001341136121PW_BS000113337116121PW_BS00002832914121PW_BS00002834524121PW_BS0000283583912PW_BS0000283683601PW_BS00002836912601PW_BS000028406351PW_BS0001153841251PW_BS0001004182451PW_BS0001153821451PW_BS0001001203171PW_BS00012012112171PW_BS0001214436171PW_BS000115448116171PW_BS00011545424171PW_BS00011539914171PW_BS0001134793101PW_BS00011548012101PW_BS00011548924101PW_BS00011548414101PW_BS000115501361PW_BS0001153911261PW_BS000112207661PW_BS000024209106PW_BS0000245062461PW_BS0001153891461PW_BS00011216212PW_BS000016204111PW_BS00002015111PW_BS000015331811PW_BS0000332441011PW_BS00002429817101PW_BS00002430013101PW_BS0000242941141PW_BS00002412915121PW_BS00012934141121PW_BS0000281141112PW_BS00011434318121PW_BS000028360410121PW_BS0000284141551PW_BS000115409115PW_BS0001154151851PW_BS00011543441051PW_BS00011545015171PW_BS0001151371117PW_BS00013745118171PW_BS000115469410171PW_BS0001154781010PW_BS0001154831110PW_BS000115208116PW_BS0000245041861PW_BS00011551541061PW_BS0001153951361PW_BS000113432511PW_BS0000437028511PW_BS000070101531PW_BS000101100521PW_BS0001001901118PW_BS0000242771218PW_BS0000242905491PW_BS0000243331212PW_BS00002835625121PW_BS000028412125PW_BS0001154192551PW_BS0001154461217PW_BS00011545525171PW_BS00011549025101PW_BS0001155072561PW_BS000115541315PW_BS00005460251PW_BS00006046114PW_BS00004672513PW_BS000072612517PW_BS0000613772113PW_BS00003793252011PW_BS00009327151PW_BS000027711PW_BS000007971521PW_BS000097105113PW_BS000105110231PW_BS000110140103PW_BS00014014315191PW_BS0001431465191PW_BS000146951721PW_BS0000951572241PW_BS00015716611PW_BS0001661802211PW_BS00018015284PW_BS00015221425181PW_BS0000242811251PW_BS0000242851041PW_BS0000242875341PW_BS00002465111PW_BS0000653081011PW_BS000024253541PW_BS00002434713125PW_BS0000283522512PW_BS00002835325127PW_BS0000283702601PW_BS000028228361PW_BS000024232403PW_BS0000244251355PW_BS000115436255PW_BS00011546013175PW_BS0001154712517PW_BS00011547225177PW_BS00011548718101PW_BS0001155131761PW_BS000115801111PW_BS0000804301151PW_BS00011546511171PW_BS0001151924Vitamin K1HMDB0003555Vitamin K1 or Phylloquinone is a polycyclic aromatic ketone, based on 1,4-naphthoquinone, with 2-methyl and 3-phytyl substituents. Vitamin K is a family of phylloquinones that contains a ring of 2-methyl-1,4-naphthoquinone and an isoprenoid side chain. Several forms of vitamin K have been identified: vitamin K 1 (vitamin K 1) derived from plants, vitamin K 2 (menaquinone) from bacteria, and synthetic naphthoquinone provitamins, vitamin K 3 (menadione). Vitamin K1 has only one double bond on the proximal isoprene unit. Vitamin K1 possesses the same type and degree of activity as does naturally-occurring vitamin K, which is necessary for the production via the liver of active prothrombin (factor II), proconvertin (factor VII), plasma thromboplastin component (factor IX), and Stuart factor (factor X). Rich sources of vitamin K 1 include green plants, algae, and photosynthetic bacteria. Vitamin K1 has antihemorrhagic and prothrombogenic activity. Vitamin K1 is a fat-soluble vitamin that is stable to air and moisture but decomposes in sunlight. It is found naturally in a wide variety of green plants.84-80-0C0205952804835839723-HYDROXY-2-METHYL-3-PHYTYL-23-DIHYDRONA4444124DB01022CC(C)CCCC(C)CCCC(C)CCC\C(C)=C\CC1=C(C)C(=O)C2=CC=CC=C2C1=OC31H46O2InChI=1S/C31H46O2/c1-22(2)12-9-13-23(3)14-10-15-24(4)16-11-17-25(5)20-21-27-26(6)30(32)28-18-7-8-19-29(28)31(27)33/h7-8,18-20,22-24H,9-17,21H2,1-6H3/b25-20+MBWXNTAXLNYFJB-LKUDQCMESA-N2-methyl-3-[(2E,7R,11R)-3,7,11,15-tetramethylhexadec-2-en-1-yl]-1,4-dihydronaphthalene-1,4-dione450.6957450.349780716-6.880vitamin K00FDB0123572',3'-trans-vitamin k1;2-methyl-3-[(2e,7r,11r)-3,7,11,15-tetramethylhexadec-2-en-1-yl]naphthalene-1,4-dione;2-methyl-3-phythyl-1,4-naphthochinon;2-methyl-3-phytyl-1,4-naphthochinon;2-methyl-3-phytyl-1,4-naphthoquinone;2-methyl-3-phytyl-1,4-napthoquinone;3-phytylmenadione;Antihemorrhagic vitamin;Aqua mephyton;Aqua-mephytin;Aquamephyton;Combinal k1;Fitomenadiona;Fitomenadione;K-ject;Kativ n;Kephton;Kinadion;Konakion;Mephyton;Mono-kay;Monodion;Phyllochinon;Phyllochinonum;Phylloquinone;Phythyl-menadion;Phytomenadione;Phytomenadionum;Phytonadione;Phytonadionum;Phytylmenadione;Synthex p;Vitamin k1;A-phylloquinone;Alpha-phylloquinone;Trans-phylloquinonePW_C001924VitK116082979220336121274429123845464146NADPHHMDB0000221Nicotinamide 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.).53-57-6C000052283351216474NADPH17215925NC(=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]1OC21H30N7O17P3InChI=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/s1ACFIXJIJDZMPPO-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 acid745.4209745.091102105-2.149[(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 acid0-4FDB0219092'-(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 - reducedPW_C000146NADPH185819037781079658211883721609291615494687314793144797145310111578910859721476128159627135677911770681887103163715420572051607315213734521075592127591170819422582191518421224118121981189321112006222121501641224528612596226126482494234331543746322769112937716613277385331773943327746013077504112775111157762333680712119113164941201054071204254051204521221206161231211411251212754291214021241214833831230593761230861351232414471237121361238464641239611181240413981254724811256962971262142991265294951270092061275723881281013902089Reduced Vitamin K (phylloquinone)HMDB0004198Vitamin K is a family of fat-soluble compounds with a common chemical structure based on 2-methyl-1,4-naphthoquinone.
Phylloquinone is often called vitamin K1, phytomenadione or phytonadione. Sometimes a distinction is made with phylloquinone considered natural and phytonadione considered synthetic. A stereoisomer of phylloquinone is called vitamin k1 (note the difference in capitalization). (Wikipedia). Phylloquinone is present in food of plant origin, such as green, leafy vegetables and certain plant oils, and is the predominant form in the diet. Bacterial and other forms of vitamin K, referred to as the menaquinones, differ in structure from phylloquinone in their 3-substituted lipophilic side chain. Menaquinone-4 (MK-4), which is alkylated from menadione, is present in animal feeds or is the product of tissue-specific conversion directly from dietary phylloquinone. Vitamin K is a cofactor specific to the formation of gamma-carboxyglutamyl (Gla) residues in certain proteins, including prothrombin necessary for normal hemostatic function. The naturally occurring forms of vitamin K are quinones (i.e. phylloquinone and menaquinones) so vitamin K is reduced to the vitamin K hydroquinone prior to catalyzing the gamma-carboxylation reaction. The active site for the carboxylation reaction is on the napthoquinone ring, which is identical for all forms of vitamin K, including phylloquinone and MK-4. (PMID 16857056).84-80-0C058505284607180674447652DB01022CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC\C(C)=C\CC1=C(C)C(=O)C2=C(C=CC=C2)C1=OC31H46O2InChI=1S/C31H46O2/c1-22(2)12-9-13-23(3)14-10-15-24(4)16-11-17-25(5)20-21-27-26(6)30(32)28-18-7-8-19-29(28)31(27)33/h7-8,18-20,22-24H,9-17,21H2,1-6H3/b25-20+/t23-,24-/m1/s1MBWXNTAXLNYFJB-NKFFZRIASA-N2-methyl-3-[(2E,7R,11R)-3,7,11,15-tetramethylhexadec-2-en-1-yl]naphthalene-1,4-diol450.6957450.349780716-6.982vitamin K hydroquinone00FDB023333Vitamin k hydroquinone (phylloquinone);2-methyl-3-(3,7,11,15-tetramethyl-2-hexadecenyl)-1,4-naphthalenedione;2-methyl-3-[(2e)-3,7,11,15-tetramethyl-2-hexadecenyl]naphthoquinone;2-methyl-3-phytyl-1,4-naphthochinon;2-methyl-3-phytyl-1,4-naphthoquinone;3-phytylmenadione;Alpha-phylloquinone;Fitomenadiona;Phyllochinon;Phyllochinonum;Phytomenadione;Phytomenadionum;Phytonadione;Phytonadionum;Phytylmenadione;Trans-phylloquinone;Vitamin k1PW_C002089ReVitK4962614713116102979221336121276429123847464143NADPHMDB0000217Nicotinamide 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).53-59-8C00006588618009NAD(P)5675NC(=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]1OC21H29N7O17P3InChI=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/s1XJLXINKUBYWONI-NNYOXOHSSA-O1-[(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-ylium744.4129744.083277073-2.2791-[(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-ylium1-3FDB021908Adenine-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 acidPW_C000143NADP1838191376857801082418839216112916174946853147961448011453081115790108601714761321596273356778117706918871051637152205720616073172137346210756221275891708197225822015184192241181119811897211120082221215216412249286125972261265024942344315437453227691329377164132773843317739633277461130775151157762433677814334778701128071311911316594120106407120429405120450122120604408120618123121142125121277429121401124121485383123063376123084135123229374123243447123713136123848464123960118124043398125473481125694297125743482126215299126528495127010206127225502127570388128100390964FADHMDB0001248FAD, 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).146-14-5C0001664397516238FAD559059DB03147CC1=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=N2C27H33N9O15P2InChI=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/s1VWWQXMAJTJZDQX-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 acid785.5497785.157134455-2.279flavine-adenine dinucleotide0-3FDB0225111h-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;AdeflavinPW_C000964FAD999114518681923216425317628288251884021188141489421612291622492133582536223723264602364688314741134758104881652681035285102533511154961265511127561311860301556054156608216161161626390164751786499179666610770391637175205732121374652227487223907622411818216118872151189921112296225123282491244315112519227125952261271029112720292130293011304130243623318770802937712613377152134775011137750711277518115775413347761513277726337780543297837534578930331792223367927235880012368800343698071411911995840611999938412005140812010740712043240512045312212049012412127842912129841812141738212148938312274812012277612112280237412282344312306637612308713512316644812384946412386845412397639912404739812534847912537848012542948212547448112569729712597948912610729912627748412689150112692039112696850212698720712701120612731020912743250612760238812784038940101Oxidized dithiothreitolHMDB0059664Oxidized dithiothreitol, also known as dithiane diol, belongs to the class of organic compounds known as dithianes. Dithianes are compounds containing a dithiane moiety, which is composed of a cyclohexane core structure wherein two methylene units are replaced by sulfur centres. Oxidized dithiothreitol is soluble (in water) and a very weakly acidic compound (based on its pKa). In humans, oxidized dithiothreitol is involved in the vitamin K metabolism pathway. Oxidized dithiothreitol is part of the Ubiquinone and other terpenoid-quinone biosynthesis pathway. It is a substrate for: Vitamin K epoxide reductase complex subunit 1.51621-02-4C0111943940742147388524DB01822O[C@H]1CSSC[C@@H]1OC4H8O2S2InChI=1S/C4H8O2S2/c5-3-1-7-8-2-4(3)6/h3-6H,1-2H2/t3-,4-/m0/s1YPGMOWHXEQDBBV-IMJSIDKUSA-N(4R,5R)-1,2-dithiane-4,5-diol152.235151.99657088-0.332dithiane diol00PW_C040101Oxidith455918792231301212801251238511361746Vitamin K1 2,3-epoxideHMDB0002972Vitamin K1 2,3-epoxide is a vitamin K derivative. Vitamin K is needed for the posttranslational modification of certain proteins, mostly required for blood coagulation. Within the cell, vitamin K undergoes electron reduction to a reduced form of vitamin K (called vitamin K hydroquinone) by the enzyme vitamin K epoxide reductase (or VKOR). Another enzyme then oxidizes vitamin K hydroquinone to allow carboxylation of glutamate into gamma-carboxyglutamate (Gla). This enzyme is called the gamma-glutamyl carboxylase or the vitamin K-dependent carboxylase. The carboxylation reaction will only proceed if the carboxylase enzyme is able to oxidize vitamin K hydroquinone into vitamin K epoxide at the same time; the carboxylation and epoxidation reactions are said to be coupled reactions. Vitamin K epoxide is then re-converted into vitamin K by the vitamin K epoxide reductase. These two enzymes comprise the so-called vitamin K cycle. One of the reasons why vitamin K is rarely deficient in a human diet is because vitamin K is continually recycled in our cells. Vitamin K 2,3-epoxide is the substrate for vitamin K 2,3-epoxide reductase (VKOR) complex. Significantly increased level of serum vitamin K epoxide has been found in patients with familial multiple coagulation factor deficiency (PMID: 12384421). Accumulation of vitamin K1-2,3-epoxide in plasma is also a sensitive marker of coumarin-like activity of drugs (PMID: 2401753).
25486-55-9C05849915772837123-EPOXY-23-DIHYDRO-2-METHYL-14-NAPHTHOQ82688CC(C)CCCC(C)CCCC(C)CCC\C(C)=C\CC12OC1(C)C(=O)C1=C(C=CC=C1)C2=OC31H46O3InChI=1S/C31H46O3/c1-22(2)12-9-13-23(3)14-10-15-24(4)16-11-17-25(5)20-21-31-29(33)27-19-8-7-18-26(27)28(32)30(31,6)34-31/h7-8,18-20,22-24H,9-17,21H2,1-6H3/b25-20+KUTXFBIHPWIDJQ-LKUDQCMESA-N(1aS,7aR)-1a-methyl-7a-[(2E,7R,11R)-3,7,11,15-tetramethylhexadec-2-en-1-yl]-1aH,2H,7H,7aH-naphtho[2,3-b]oxirene-2,7-dione466.706466.344695341-7.320(1aS,7aR)-1a-methyl-7a-[(2E,7R,11R)-3,7,11,15-tetramethylhexadec-2-en-1-yl]naphtho[2,3-b]oxirene-2,7-dione00FDB023087(2,3-epoxyphytyl)menaquinone;1,4-naphthoquinone, 2,3-epoxy-2,3-dihydro-2-methyl-3-phytyl-2,3-epoxyphylloquinone;2,3-epoxy-2,3-dihydro-2-methyl-3-phytyl-1,4-naphthoquinone;2,3-epoxyphylloquinone;Naphth[2,3-b]oxirene-2,7-dione, 1a,7a-dihydro-1a-methyl-7a-(3,7,11,15-tetramethyl-2-hexadecenyl)-phylloquinone oxide;Phylloquinone 2,3-epoxide;Phylloquinone-2,3-epoxide;Vitamin k 2,3-epoxide;Vitamin k epoxide;Vitamin k1 oxide;1a,7a-dihydro-1a-methyl-7a-(3,7,11,15-tetramethyl-2-hexadecenyl)-naphth[2,3-b]oxirene-2,7-dione;Phylloquinone epoxide;Phylloquinone oxide;Phylloquinone, epoxide;Vitamin k1 2,3-epoxide;Vitamin k1, epoxidePW_C001746VitKo495261472311661294560187922433612128142912385246485091,4-DithiothreitolHMDB0013593Dithiothreitol (DTT) is the common name for a small-molecule redox reagent known as Cleland's reagent. DTT's formula is C4H10O2S2 and the molecular structure of its reduced form is shown at the right; its oxidized form is a disulfide-bonded 6-membered ring (shown below). Its name derives from the four-carbon sugar, threose. DTT has an epimeric ('sister') compound, dithioerythritol. A common use of DTT is as a reducing or "deprotecting" agent for thiolated DNA. The terminal sulfur atoms of thiolated DNA have a tendency to form dimers in solution, especially in the presence of oxygen. Dimerization greatly lowers the efficiency of subsequent coupling reactions such as DNA immobilization on gold in biosensors. Typically DTT is mixed with a DNA solution and allowed to react, and then is removed by filtration (for the solid catalyst) or by chromatography (for the liquid form). The DTT removal procedure is often called "desalting.". DTT is frequently used to reduce the disulfide bonds of proteins and, more generally, to prevent intramolecular and intermolecular disulfide bonds from forming between cysteine residues of proteins. However, even DTT cannot reduce buried (solvent-inaccessible) disulfide bonds, so reduction of disulfide bonds is sometimes carried out under denaturing conditions (e.g., at high temperatures, or in the presence of a strong denaturant such as 6 M guanidinium hydrochloride, 8 M urea, or 1% sodium dodecylsulfate). Conversely, the solvent exposure of different disulfide bonds can be assayed by their rate of reduction in the presence of DTT. DTT can also be used as an oxidizing agent. Its principal advantage is that effectively no mixed-disulfide species are populated, in contrast to other agents such as glutathione. In very rare cases, a DTT adduct may be formed, i.e., the two sulfur atoms of DTT may form disulfide bonds to different sulfur atoms; in such cases, DTT cannot cyclize since it has no remaining free thiols. Due to air oxidation, DTT is a relatively unstable compound whose useful life can be extended by refrigeration and handling in an inert atmosphere. Since protonated sulfurs have lowered nucleophilicities, DTT becomes less potent as the pH lowers. Tris(2-carboxyethyl)phosphine HCl (TCEP hydrochloride) is an alternative which is more stable and works even at low pH.3483-12-3C0026543919642106388336O[C@@H](CS)[C@@H](O)CSC4H10O2S2InChI=1S/C4H10O2S2/c5-3(1-7)4(6)2-8/h3-8H,1-2H2/t3-,4-/m0/s1VHJLVAABSRFDPM-IMJSIDKUSA-N(2R,3R)-1,4-disulfanylbutane-2,3-diol154.251154.012220944-1.484L-dithiothreitol00C00265(2r,3r)-1,4-dimercaptobutane-2,3-diol;1,4-dithiothreitol;2,3-dihydroxy-1,4-dithiobutane;Dl-threo-1,4-dimercapto-2,3-butanediol;L-dtt;L-threo-1,4-dimercapto-2,3-butanediol;Threo-1,4-dimercapto-2,3-butanediolPW_C008509L-DTT456118792251301212821251238531361065OxygenHMDB0001377Oxygen is the third most abundant element in the universe after hydrogen and helium and the most abundant element by mass in the Earth's crust. Diatomic oxygen gas constitutes 20.9% of the volume of air. All major classes of structural molecules in living organisms, such as proteins, carbohydrates, and fats, contain oxygen, as do the major inorganic compounds that comprise animal shells, teeth, and bone. Oxygen in the form of O2 is produced from water by cyanobacteria, algae and plants during photosynthesis and is used in cellular respiration for all living organisms. Green algae and cyanobacteria in marine environments provide about 70% of the free oxygen produced on earth and the rest is produced by terrestrial plants. Oxygen is used in mitochondria to help generate adenosine triphosphate (ATP) during oxidative phosphorylation. For animals, a constant supply of oxygen is indispensable for cardiac viability and function. To meet this demand, an adult human, at rest, inhales 1.8 to 2.4 grams of oxygen per minute. This amounts to more than 6 billion tonnes of oxygen inhaled by humanity per year. At a resting pulse rate, the heart consumes approximately 8-15 ml O2/min/100 g tissue. This is significantly more than that consumed by the brain (approximately 3 ml O2/min/100 g tissue) and can increase to more than 70 ml O2/min/100 g myocardial tissue during vigorous exercise. As a general rule, mammalian heart muscle cannot produce enough energy under anaerobic conditions to maintain essential cellular processes; thus, a constant supply of oxygen is indispensable to sustain cardiac function and viability. However, the role of oxygen and oxygen-associated processes in living systems is complex, and they and can be either beneficial or contribute to cardiac dysfunction and death (through reactive oxygen species). Reactive oxygen species (ROS) are a family of oxygen-derived free radicals that are produced in mammalian cells under normal and pathologic conditions. Many ROS, such as the superoxide anion (O2-)and hydrogen peroxide (H2O2), act within blood vessels, altering mechanisms mediating mechanical signal transduction and autoregulation of cerebral blood flow. Reactive oxygen species are believed to be involved in cellular signaling in blood vessels in both normal and pathologic states. The major pathway for the production of ROS is by way of the one-electron reduction of molecular oxygen to form an oxygen radical, the superoxide anion (O2-). Within the vasculature there are several enzymatic sources of O2-, including xanthine oxidase, the mitochondrial electron transport chain, and nitric oxide (NO) synthases. Studies in recent years, however, suggest that the major contributor to O2- levels in vascular cells is the membrane-bound enzyme NADPH-oxidase. Produced O2- can react with other radicals, such as NO, or spontaneously dismutate to produce hydrogen peroxide (H2O2). In cells, the latter reaction is an important pathway for normal O2- breakdown and is usually catalyzed by the enzyme superoxide dismutase (SOD). Once formed, H2O2 can undergo various reactions, both enzymatic and nonenzymatic. The antioxidant enzymes catalase and glutathione peroxidase act to limit ROS accumulation within cells by breaking down H2O2 to H2O. Metabolism of H2O2 can also produce other, more damaging ROS. For example, the endogenous enzyme myeloperoxidase uses H2O2 as a substrate to form the highly reactive compound hypochlorous acid. Alternatively, H2O2 can undergo Fenton or Haber-Weiss chemistry, reacting with Fe2+/Fe3+ ions to form toxic hydroxyl radicals (-.OH). (PMID: 17027622, 15765131).7782-44-7C0000797715379CPD-6641952O=OO2InChI=1S/O2/c1-2MYMOFIZGZYHOMD-UHFFFAOYSA-Noxidanone31.998831.9898292440singlet oxygen00FDB022589Dioxygen;Molecular oxygen;O2;Oxygen;Oxygen molecule;[oo];Dioxygene;Disauerstoff;E 948;E-948;E948PW_C001065O2959110524516500185058549146252863836491067431688207541576347693383621375492016242531222803294260424747135467123548012554931265508127580910859731476129159700618870321637050160731921375332107560212839515111816216118641981188321511894211120572251206316412247286122792261232524912706291127162921300429813016300130263011303830213260223422761742657315769102937704429477214134773501117736313077377331773953327749711377512115775373347762633677723337777361127774712977756341778051147781213378070329781511327838134578805343791113601200474081203831221204264051205424071205534141205944091206014061208834151210451241211043831216054341216564291221173821225734181226893841227983741228224431230271351230603761231284471231391361231634481231761191231874501232191371232261201234594511236091181236693981241634691242144641246693991251454541252751211254254821257064781257314831257372971257404791258844811261002991262724841265224951267214891268254801269645021269862071271982091272142081272192051272225011273055041273452061275573881275745151278353891280813951280953901283125061284323911316Carbon 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_C001316CO250812112044480135031864036773169520806511334316384917452255117314470528310353201115750108577110159681006026155607816164711786637107692219070171607035163706118871632057308198733321374612227530210821522582231519158249118492771190817012464226126882904262631543523318769942937712213377170132774703337773911277750129777633417807713478405356784273347894133179227130800083688067511980717135948363841132913911155491211199544061200891221201554071203644121205564141208334191209221241209914081212841251215053831227441201230114461231904501234184551234891181235563741238551361240633981253444791254602971255164811258244901258702991259314821262804801268875011270522061272775071273313881273905021420WaterHMDB0002111Water 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).7732-18-5C0000196215377937OH2OInChI=1S/H2O/h1H2XLYOFNOQVPJJNP-UHFFFAOYSA-Nwater18.015318.0105646861water00FDB013390Dihydrogen oxide;Steam;[oh2];Acqua;Agua;Aqua;Bound water;Dihydridooxygen;Eau;H2o;Hoh;Hydrogen hydroxide;WasserPW_C001420H2O5589491095139415131621448113526156242865210691207703382318838210943113774914655415904320182425322226786027274627781728052931437031647236346145983647273749419350302751567519597521410052279452361035297105531911153431135355112540211054701235483125549212655071275534130553711455411295591135560811856221085691657591405778101584114358531465877107589095591014759401516032155605915760871616123163613315962151621816664771786507180660015267131176840188688816071622057181207719320672112117228213723821472432157295198735021673882107401212746722274922247500190758817082012258237226841416292652611850277119221641201128112213285122502861226428712327249125202271263265126932901270529112715292130072981301930013025301130373021326122313327294153403084232731542695318436913227691429377019253771021327713113377215134773783317739733277471333775161157753633477628336777223377775934177816343779823477807132978235352782423537827035679113360800143688003937080591228806561199383038394794384110557390110639391115844398119879232119915122119963406120008407120046408120113124120365412120430405120438409120606415120794414121158425121240429121351121121381419121607434122118382122384436122753120122797374122804443123012446123064376123072137123131447123142136123162448123231451123384450123730460123810464123940455124165469124670399124938471124945472125305297125353479125386481125424482125480299125682483125707478125745487126054490126238495126273484126764480126896501126963502127017388127177208127199209127227504127506507127576515127836389128082395128176513159Propionic acidHMDB0000237Propionic acid (PA) is widely used as an antifungal agent in food. It is present naturally at low levels in dairy products and occurs ubiquitously, together with other short-chain fatty acids (SCFA), in the gastro-intestinal tract of humans and other mammals as an end-product of the microbial digestion of carbohydrates. It has significant physiological activity in animals. PA is irritant but produces no acute systemic effects and has no demonstrable genotoxic potential. (PMID 1628870) Propionic aciduria is one of the most frequent organic acidurias, a disease that comprise many various disorders. The outcome of patients born with Propionic aciduria is poor intellectual development patterns, with 60% having an IQ less than 75 and requiring special education. Successful liver and/or renal transplantations, in a few patients, have resulted in better quality of life but have not necessarily prevented neurological and various visceral complications. These results emphasize the need for permanent metabolic follow-up whatever the therapeutic strategy. (PMID 15868474) Decreased early mortality, less severe symptoms at diagnosis, and more favorable short-term neurodevelopmental outcome were recorded in patients identified through expanded newborn screening. (PMID 16763906).79-09-4C00163103230768PROPIONATE1005DB03766CCC(O)=OC3H6O2InChI=1S/C3H6O2/c1-2-3(4)5/h2H2,1H3,(H,4,5)XBDQKXXYIPTUBI-UHFFFAOYSA-Npropanoic acid74.078574.0367794360.681propanoic acid0-1FDB008285Adofeed;Antischim b;Carboxyethane;Ethanecarboxylate;Ethanecarboxylic acid;Ethylformate;Ethylformic acid;Luprosil;Metacetonate;Metacetonic acid;Methylacetate;Methylacetic acid;Monoprop;Propanate;Propanoate;Propanoic acid;Propcorn;Propionate;Propkorn;Prozoin;Pseudoacetate;Pseudoacetic acid;Toxi-check;Acide propanoique;Acide propionique;Ch3-ch2-cooh;Pa;Propioic acid;Propionsaeure;Propoic acid;Propioate;PropoatePW_C0001593:0230744563186363107636410842468318424693157863913379228130121285125122272406123856136124825120126434479128003501130Methylmalonic acidHMDB0000202Methylmalonic acid is a malonic acid derivative, which is a vital intermediate in the metabolism of fat and protein. In particular, the coenzyme A-linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase in a reaction that requires vitamin B12 as a cofactor. In this way, methylmalonic acid enters the Krebs cycle and is thus part of one of the anaplerotic reactions. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This inborn error of metabolism is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. Methylmalonic acid is also found to be associated with other inborn errors of metabolism, including cobalamin deficiency, cobalamin malabsorption, malonyl-CoA decarboxylase deficiency, and transcobalamin II deficiency. When present in sufficiently high levels, methylmalonic acid can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of methylmalonic acid are associated with at least 5 inborn errors of metabolism, including Malonyl CoA decarboxylase deficiency, Malonic Aciduria, Methylmalonate Semialdehyde Dehydrogenase Deficiency, Methylmalonic Aciduria and Methylmalonic Aciduria Due to Cobalamin-Related Disorders. Methylmalonic acid is an organic acid and abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of the untreated IEMs mentioned above. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.516-05-2C0217048730860473DB04183CC(C(O)=O)C(O)=OC4H6O4InChI=1S/C4H6O4/c1-2(3(5)6)4(7)8/h2H,1H3,(H,5,6)(H,7,8)ZIYVHBGGAOATLY-UHFFFAOYSA-N2-methylpropanedioic acid118.088118.026608680.102methylmalonic acid0-2FDB0219051,1-ethanedicarboxylate;1,1-ethanedicarboxylic acid;2-methylmalonate;2-methylmalonic acid;Isosuccinate;Isosuccinic acid;Methyl-malonate;Methyl-malonic acid;Methyl-propanedioate;Methyl-propanedioic acid;Methylmalonate;Methylmalonic acid;Methylpropanedioate;Methylpropanedioic acid;Alpha-methylmalonic acid;2-methylpropanedioate;A-methylmalonate;A-methylmalonic acid;Alpha-methylmalonate;α-methylmalonate;α-methylmalonic acidPW_C000130Isosca1683823164456518456724569157862713379205111792291307923113279232114826778012128712512128943012158412212225640612385813612386046512414213512480912012641847912798450116191NAD(P)H dehydrogenase [quinone] 1Q63478
The enzyme apparently serves as a quinone reductase in connection with conjugation reactions of hydroquinons involved in detoxification pathways as well as in biosynthetic processes such as the vitamin K-dependent gamma-carboxylation of glutamate residues in prothrombin synthesis.
Nqo1171.6.5.212385046416194Vitamin K epoxide reductase complex subunit 1Q6TEK4
Involved in vitamin K metabolism. Catalytic subunit of the vitamin K epoxide reductase (VKOR) complex which reduces inactive vitamin K 2,3-epoxide to active vitamin K. Vitamin K is required for the gamma-carboxylation of various proteins, including clotting factors, and is required for normal blood coagulation, but also for normal bone development.
Vkorc1171.17.4.412385413613261846413261944716198ProthrombinP18292
Thrombin, which cleaves bonds after Arg and Lys, converts fibrinogen to fibrin and activates factors V, VII, VIII, XIII, and, in complex with thrombomodulin, protein C. Functions in blood homeostasis, inflammation and wound healing.
F2173.4.21.512385713612386146513262013716203Vitamin K-dependent gamma-carboxylaseO88496
Mediates the vitamin K-dependent carboxylation of glutamate residues to calcium-binding gamma-carboxyglutamate (Gla) residues with the concomitant conversion of the reduced hydroquinone form of vitamin K to vitamin K epoxide.
Ggcx174.1.1.901238591361342004479101NAD(P)H dehydrogenase [quinone] 117PW_P009101173541619129102Vitamin K epoxide reductase complex subunit 117PW_P00910217355161949103[Prothrombin]-Propionic acid17PW_P009103173561619819104[Prothrombin]-Methylmalonic acid17PW_P009104173571619819105Vitamin K-dependent gamma-carboxylase17PW_P0091051735816203147376falsePW_R147376Right54944519241Compoundfalse5494461461Compoundtrue54944720891Compoundfalse5494481431Compoundtrue13761891011.6.5.2147377falsePW_R147377Right54944919241Compoundfalse549450401011Compoundtrue54945117461Compoundfalse54945285091Compoundtrue13761991021.1.4.1147378falsePW_R147378Right54945320891Compoundfalse54945491031ProteinComplexfalse54945510651Compoundtrue54945613161Compoundtrue54945717461Compoundfalse54945891041ProteinComplexfalse54945914201Compoundtrue13762091054.1.1.908027PW_T008027828491041ProteinComplex136465Right2660731192446482false22572010regular300280266073214646462false69074010regular50302660733208946482false114572010regular300280266073414346461false102074010regular503026607359644649false81582010regular100252660736401011363false155131010regular1001102660737174646482false645148510regular300280266073885091363false465146510regular1001102660739106513665false961116010regular78782660740131613652false981106310regular78782660741142013649false985145010regular787826607421591369false1255114010regular1002526607431301369false1145165510regular1003526607441304659false1145194210regular10035929606161914646false7858258subunitregular16080929607161941362false45513278subunitregular15070929608161981362false123011558subunitregular15070929609161981362false105516808subunitregular15070929610162031362false106013248subunitregular15070929611161984652false105519628subunitregular150707939809101880964649260669296063277126607353623876Cofactor7939819102880961369260679296077939829103880961369260689296083277226607423623886Cofactor7939839104880961369260699296093277326607433623888Cofactor7939849105880961369260709296107939859104880964659260719296113277426607443623891Cofactor3623872M525 860 C555 860 755 865 785 865 5false183623873M715 770 C715 825 735 866 785 865 5false183623874M1145 860 C1115 860 975 865 945 865 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3623875M1045 770 C1034 848 975 865 945 865 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3623876M810 715 L880 810 L918 712 z10true183623877M375 1000 C245 1078 211 1303 455 1362 5false183623878M255 1365 C312 1364 401 1363 455 1362 5false183623879M795 1485 C795 1455 767 1371 605 1362 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3623880M565 1520 C640 1498 675 1383 605 1362 5false18trueM 25.946855044164835 13.26155629629604 L 11 12 L 17.380887721185843 25.575134323078345false3623881M1295 1000 C1134 1062 1135 1294 1135 1324 5false183623882M1039 1199 C1156 1188 1118 1287 1135 1324 5false183623883M1059 1102 C1065 1111 1138 1085 1135 1324 5false183623884M945 1625 C1134 1575 1135 1424 1135 1394 5false18trueM 236.94685504416483 1139.261556296296 L 222 1138 L 228.38088772118584 1151.5751343230784false3623885M1063 1489 C1131 1489 1135 1424 1135 1394 5false18trueM 236.94685504416483 1139.261556296296 L 222 1138 L 228.38088772118584 1151.5751343230784false3623886M1250 1135 L1250 1185 L1300 1135 z10true183623887M1230 1190 C1152 1198 1136 1294 1135 1324 5false183623888M1075 1650 L1075 1700 L1125 1650 z10true183623889M1130 1680 C1159 1585 1135 1424 1135 1394 5false18trueM 236.94685504416483 1139.261556296296 L 222 1138 L 228.38088772118584 1151.5751343230784false3623890M1130 1750 C1130 1780 1131 1738.25 1131 1768.25 83false183623891M1075 1697.5 L1075 1747.5 L1125 1697.5 z10true183623892M1130 1962 C1130 1932 1130 1781.25 1130 1751.25 83false18trueM 239.94685504416483 1320.261556296296 L 225 1319 L 231.38088772118584 1332.5751343230784false79189388096147376464320525226607313623872Left320525326607323623873Left320525426607333623874Right320525526607343623875Right74837713761879398079189488096147377136320525626607313623877Left320525726607363623878Left320525826607373623879Right320525926607383623880Right74837813761979398179189588096147378136320526026607333623881Left320526126607393623882Left320526226607403623883Left320526326607373623884Right320526426607413623885Right166477939823623887Left166487939833623889Right748379137620793984770338027880965437939833623890Left5447939853623892Right18502572380302.82.8029200145185026664959500.90.9023280360368510M363 1362 C446 1364 1206 1356 1283 1357 91false6920.05.0368511M87 648 C87 598 137 548 187 548 C565 548 1057 548 1435 548 C1485 548 1535 598 1535 648 C1535 990 1535 1434 1535 1776 C1535 1826 1485 1876 1435 1876 C1057 1876 565 1876 187 1876 C137 1876 87 1826 87 1776 C87 1434 87 990 87 648 1true61448.01328.044727215Liver650280201.01.01601544727315Intracellular SPace160600201.01.01601544727415Extracellular Space85355201.01.01601544727515Endoplasmic reticulum6351015201.01.0160151798835143413349515882062441555156717988461712013631308129318014#FFEEDE4930493