Metabolism of water-soluble vitamins and cofactors (Homo sapiens)

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77248, 386620, 6919, 3623, 654732, 56412, 4212, 4212, 422237412, 4262829, 41, 58, 6246, 5211, 549923, 6537, 5925, 5948, 7315, 5746, 5253, 752217, 29, 41551426, 27, 40, 43, 6333, 591, 2, 31, 50, 60...46, 52515, 21, 64, 6761025, 5937, 591429, 41, 45, 493034, 5113, 44, 7015, 5716, 7133, 593539, 5453, 757612, 4261, 631833, 59mitochondrial matrixnucleoplasmcytosolGolgi lumenATPCoA-SHNADP+H2OH2OH2O2GlutathioneTHF-polyglutamateAMP5,10-methylenetetrahydrofolate polyglutamateH2OADPNicotinate phosphoribosyltransferase homodimerATPThiamin triphosphateATPTRAP homodimerATPADPDihydrofolate reductaseNMNAT3 tetramerPantothenateMOCS3(Zn++) (ox.)PRPPsulfurated MoCosodium ionFolylpolyglutamate synthase-1BiotinADPMoCodehydroascorbateH+Folylpolyglutamate synthase-2H2OPyridoxaminePyridoxamine phosphateCYB5R3:FADNADHsodium ionAMPH+H+NADPHferric CYB5ACO2MOCOS holoenzymeTetrahydrofolateFlavin mononucleotideNicotinate D-ribonucleotideNADP+FAD1 flavin adenine dinucleotide synthetase homologMOCS2 tetramerDehydroascorbateSHMT1 tetramerOrthophosphatePrecursor ZFADSLC19A1ATPNADP+Deamino-NAD+PhosphopantothenoylcysteineOrthophosphateSLC19A2; SLC19A3L-serineMagnesiumpyrophosphateL-GlutamateADPDephospho-CoA5-methyltetrahydrofolate polyglutamateGLUT1; GLUT3FolateL-GlutamineSLC25A32ThiaminFatty acid synthase apoenzymesemidehydroascorbateAMPATPPDXK holoenzymepyrophosphateOrthophosphatePPC synthase homodimerH+Thiamin diphosphateSMVTpyrophosphateAMPQuinolinateH+NADP+PPCDC homotrimer (FMN cofactor)PyridoxalADPFormateBifunctional coenzyme A synthaseThDP kinaseADPPhospho-pantothenateH2OL-AlanineNamPRTThiaminSLC25A16MTHFD1 dimerNAD+MOCS3(Zn++) (red.)Nicotinamide deaminaseMOCS1 holoenzymepyrophosphatesodium ionPantothenate kinase 2CoA-SHpyrophosphateNADPHNicotinateATPATPNAD+H+pyrophosphatepyrophosphateAMPPNPO holoenzymeADPL-GlutamateATPNAD+ kinase homotetramer (Zn2+)glycineTHTPA-Mg++Fatty acid synthaseAscorbateATPGSTO homodimersMOCS2-CO-S(1-) tetramerATPGephyrin(Mg++) trimerADPOxygenCO2Deamino-NAD+2-Amino-3-carboxymuconate semialdehydepyrophosphateOrthophosphateTetrahydrofolateL-cysteineSLC46A1Riboflavin10-formyltetrahydrofolate polyglutamatePantothenateATPGTPADPascorbatemolybdate(2-)5'-DeoxyadenosinepyrophosphateNH4+OrthophosphateDihydrofolateATPPyridoxineSVCT1; SVCT2H2O5-methyltetrahydrofolateL-cysteineNADPHH2OH2OTPK1 homodimer (Mg2+)Nicotinate D-ribonucleotideNADSYN1 homohexamerATPATPNFS1 holoenzymeH2OS-adenosylmethionineMTHFR dimerATPL-AlanineADPMolybdopterinNH3AMP5,10-methenyltetrahydrofolate polyglutamateMOCS2ARiboflavin kinase (Mg2+)pyrophosphatesodium ionATPH+MOCS3(Zn++)-S-S(1-)THF-polyglutamateADPPyridoxine phosphateATPNMNAT1 hexamerMOCS2A-CO-S(1-)Nicotinate D-ribonucleotidePRPPFolateNicotinamidePAPH2ONucleotide pyrophosphatase homodimerL-cysteinePANK1;3;4pyrophosphatePyridoxal phosphateNicotinate D-ribonucleotide5-methyltetrahydrofolateferrous CYB5AH+oxidized glutathioneBiotinNMNAT2 (Mg2+)L-GlutamateH2O4' phosphopantetheinePRPPpyrophosphateNicotinate-nucleotide pyrophosphorylaseATPL-MethionineAASDHPPT7


Vitamins are a diverse group of organic compounds, required in small amounts in the diet. They have distinct biochemical roles, often as coenzymes, and are either not synthesized or synthesized only in limited amounts by human cells. Vitamins are classified according to their solubility, either fat-soluble or water-soluble. The physiological processes dependent on vitamin-requiring reactions include many aspects of intermediary metabolism, vision, bone formation, and blood coagulation, and vitamin deficiencies are associated with a correspondingly diverse and severe group of diseases.

Water-soluble vitamins include ascorbate (vitamin C) and the members of the B group: thiamin (vitamin B1), riboflavin (B2), niacin (B3), pantothenate (B5), pyridoxine (B6), biotin (B7), folate (B9), and cobalamin (B12). Metabolic processes annotated here include the synthesis of thiamin pyrophosphate (TPP) from thiamin (B1), the synthesis of FMN and FAD from riboflavin (B2), the synthesis of nicotinic acid (niacin - B3) from tryptophan, the synthesis of Coenzyme A from pantothenate (B5), and features of the metabolism of folate (B9).</div>

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  110. Said HM, Balamurugan K, Subramanian VS, Marchant JS.; ''Expression and functional contribution of hTHTR-2 in thiamin absorption in human intestine.''; PubMed Europe PMC Scholia
  111. Randaccio L, Geremia S, Demitri N, Wuerges J.; ''Vitamin B12: unique metalorganic compounds and the most complex vitamins.''; PubMed Europe PMC Scholia
  112. Cole H, Reynolds TR, Lockyer JM, Buck GA, Denson T, Spence JE, Hymes J, Wolf B.; ''Human serum biotinidase. cDNA cloning, sequence, and characterization.''; PubMed Europe PMC Scholia
  113. Fyfe JC, Madsen M, Højrup P, Christensen EI, Tanner SM, de la Chapelle A, He Q, Moestrup SK.; ''The functional cobalamin (vitamin B12)-intrinsic factor receptor is a novel complex of cubilin and amnionless.''; PubMed Europe PMC Scholia
  114. Bosch AM, Abeling NG, Ijlst L, Knoester H, van der Pol WL, Stroomer AE, Wanders RJ, Visser G, Wijburg FA, Duran M, Waterham HR.; ''Brown-Vialetto-Van Laere and Fazio Londe syndrome is associated with a riboflavin transporter defect mimicking mild MADD: a new inborn error of metabolism with potential treatment.''; PubMed Europe PMC Scholia
  115. Suzuki Y, Yang X, Aoki Y, Kure S, Matsubara Y.; ''Mutations in the holocarboxylase synthetase gene HLCS.''; PubMed Europe PMC Scholia
  116. Karthikeyan S, Zhou Q, Mseeh F, Grishin NV, Osterman AL, Zhang H.; ''Crystal structure of human riboflavin kinase reveals a beta barrel fold and a novel active site arch.''; PubMed Europe PMC Scholia
  117. Fan C, Bobik TA.; ''Functional characterization and mutation analysis of human ATP:Cob(I)alamin adenosyltransferase.''; PubMed Europe PMC Scholia
  118. Ashokkumar B, Vaziri ND, Said HM.; ''Thiamin uptake by the human-derived renal epithelial (HEK-293) cells: cellular and molecular mechanisms.''; PubMed Europe PMC Scholia
  119. Diaz GA, Banikazemi M, Oishi K, Desnick RJ, Gelb BD.; ''Mutations in a new gene encoding a thiamine transporter cause thiamine-responsive megaloblastic anaemia syndrome.''; PubMed Europe PMC Scholia
  120. Wolthers KR, Scrutton NS.; ''Cobalamin uptake and reactivation occurs through specific protein interactions in the methionine synthase-methionine synthase reductase complex.''; PubMed Europe PMC Scholia
  121. Kristiansen M, Aminoff M, Jacobsen C, de La Chapelle A, Krahe R, Verroust PJ, Moestrup SK.; ''Cubilin P1297L mutation associated with hereditary megaloblastic anemia 1 causes impaired recognition of intrinsic factor-vitamin B(12) by cubilin.''; PubMed Europe PMC Scholia
  122. Kalousek F, Darigo MD, Rosenberg LE.; ''Isolation and characterization of propionyl-CoA carboxylase from normal human liver. Evidence for a protomeric tetramer of nonidentical subunits.''; PubMed Europe PMC Scholia
  123. Wuebbens MM, Rajagopalan KV.; ''Mechanistic and mutational studies of Escherichia coli molybdopterin synthase clarify the final step of molybdopterin biosynthesis.''; PubMed Europe PMC Scholia
  124. Quadros EV, Lai SC, Nakayama Y, Sequeira JM, Hannibal L, Wang S, Jacobsen DW, Fedosov S, Wright E, Gallagher RC, Anastasio N, Watkins D, Rosenblatt DS.; ''Positive newborn screen for methylmalonic aciduria identifies the first mutation in TCblR/CD320, the gene for cellular uptake of transcobalamin-bound vitamin B(12).''; PubMed Europe PMC Scholia
  125. Stallmeyer B, Schwarz G, Schulze J, Nerlich A, Reiss J, Kirsch J, Mendel RR.; ''The neurotransmitter receptor-anchoring protein gephyrin reconstitutes molybdenum cofactor biosynthesis in bacteria, plants, and mammalian cells.''; PubMed Europe PMC Scholia
  126. Coelho D, Kim JC, Miousse IR, Fung S, du Moulin M, Buers I, Suormala T, Burda P, Frapolli M, Stucki M, Nürnberg P, Thiele H, Robenek H, Höhne W, Longo N, Pasquali M, Mengel E, Watkins D, Shoubridge EA, Majewski J, Rosenblatt DS, Fowler B, Rutsch F, Baumgartner MR.; ''Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism.''; PubMed Europe PMC Scholia
  127. Srinivasan B, Baratashvili M, van der Zwaag M, Kanon B, Colombelli C, Lambrechts RA, Schaap O, Nollen EA, Podgoršek A, Kosec G, Petković H, Hayflick S, Tiranti V, Reijngoud DJ, Grzeschik NA, Sibon OC.; ''Extracellular 4'-phosphopantetheine is a source for intracellular coenzyme A synthesis.''; PubMed Europe PMC Scholia
  128. Abu-Elheiga L, Jayakumar A, Baldini A, Chirala SS, Wakil SJ.; ''Human acetyl-CoA carboxylase: characterization, molecular cloning, and evidence for two isoforms.''; PubMed Europe PMC Scholia
  129. Wolf B, Grier RE, Allen RJ, Goodman SI, Kien CL.; ''Biotinidase deficiency: the enzymatic defect in late-onset multiple carboxylase deficiency.''; PubMed Europe PMC Scholia
  130. Ichida K, Matsumura T, Sakuma R, Hosoya T, Nishino T.; ''Mutation of human molybdenum cofactor sulfurase gene is responsible for classical xanthinuria type II.''; PubMed Europe PMC Scholia
  131. Green P, Wiseman M, Crow YJ, Houlden H, Riphagen S, Lin JP, Raymond FL, Childs AM, Sheridan E, Edwards S, Josifova DJ.; ''Brown-Vialetto-Van Laere syndrome, a ponto-bulbar palsy with deafness, is caused by mutations in c20orf54.''; PubMed Europe PMC Scholia
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101226view11:12, 1 November 2018ReactomeTeamreactome version 66
100764view20:38, 31 October 2018ReactomeTeamreactome version 65
100308view19:15, 31 October 2018ReactomeTeamreactome version 64
99854view15:58, 31 October 2018ReactomeTeamreactome version 63
99412view14:35, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99099view12:39, 31 October 2018ReactomeTeamreactome version 62
94004view13:50, 16 August 2017ReactomeTeamreactome version 61
93616view11:28, 9 August 2017ReactomeTeamreactome version 61
86724view09:24, 11 July 2016ReactomeTeamreactome version 56
83083view09:55, 18 November 2015ReactomeTeamVersion54
81407view12:56, 21 August 2015ReactomeTeamVersion53
76875view08:14, 17 July 2014ReactomeTeamFixed remaining interactions
76580view11:56, 16 July 2014ReactomeTeamFixed remaining interactions
75913view09:56, 11 June 2014ReactomeTeamRe-fixing comment source
75613view10:47, 10 June 2014ReactomeTeamReactome 48 Update
74968view13:49, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74612view08:39, 30 April 2014ReactomeTeamReactome46
44904view10:24, 6 October 2011MartijnVanIerselOntology Term : 'metabolic pathway of cofactors and vitamins' added !
42173view23:38, 4 March 2011MaintBotModified categories
42075view21:55, 4 March 2011MaintBotAutomatic update
39883view05:54, 21 January 2011MaintBotNew pathway

External references


View all...
NameTypeDatabase referenceComment

formyltetrahydro folate polyglutamate

MetaboliteREACT_4874 (Reactome)

carboxymuconate semialdehyde

Metabolite29044 (ChEBI)

phosphopantet heine

Metabolite16858 (ChEBI)
5'-Deoxyadenosine Metabolite17319 (ChEBI)

methenyltetra hydrofolate polyglutamate

MetaboliteREACT_11283 (Reactome)

methylenetetrahyd rofolate polyglutamate

MetaboliteREACT_11343 (Reactome)

methyltetrahydro folate

Metabolite15641 (ChEBI)

methyltetrahydro folate polyglutamate

MetaboliteREACT_7545 (Reactome)
AASDHPPT ProteinQ9NRN7 (UniProt)
ADP Metabolite16761 (ChEBI)
AMP Metabolite16027 (ChEBI)
ATP Metabolite15422 (ChEBI)
Ascorbate Metabolite29073 (ChEBI)

coenzyme A synthase

ProteinQ13057 (UniProt)
Biotin Metabolite15956 (ChEBI)
CO2 Metabolite16526 (ChEBI)


ComplexREACT_11367 (Reactome)
CoA-SH Metabolite15346 (ChEBI)


Metabolite18304 (ChEBI)
Dehydroascorbate Metabolite17242 (ChEBI)
Dephospho-CoA Metabolite15468 (ChEBI)
Dihydrofolate Metabolite15633 (ChEBI)


ProteinP00374 (UniProt)
FAD Metabolite16238 (ChEBI)
FAD1 flavin

adenine dinucleotide synthetase homolog

ProteinQ8NFF5 (UniProt)
Fatty acid


ProteinP49327 (UniProt)
Fatty acid

synthase apoenzyme

ProteinP49327 (UniProt)

mononucleoti de

Metabolite17621 (ChEBI)
Folate Metabolite27470 (ChEBI)

amate synthase-2

ProteinQ05932-2 (UniProt)


ProteinQ05932-1 (UniProt)
Formate Metabolite30751 (ChEBI)


ProteinREACT_11567 (Reactome)


ComplexREACT_11879 (Reactome)
GTP Metabolite15996 (ChEBI)


ComplexREACT_26924 (Reactome)
Glutathione Metabolite16856 (ChEBI)
H+ Metabolite15378 (ChEBI)
H2O Metabolite15377 (ChEBI)
H2O2 Metabolite16240 (ChEBI)
L-Alanine Metabolite16977 (ChEBI)
L-Glutamate Metabolite16015 (ChEBI)
L-Glutamine Metabolite18050 (ChEBI)
L-Methionine Metabolite16643 (ChEBI)
L-cysteine Metabolite17561 (ChEBI)
L-serine Metabolite17115 (ChEBI)
MOCOS holoenzyme ComplexREACT_26664 (Reactome)


ComplexREACT_26979 (Reactome)


ComplexREACT_25714 (Reactome)


ComplexREACT_25716 (Reactome)
MOCS2A ProteinO96033 (UniProt)
MOCS2A-CO-S(1-) ProteinO96033 (UniProt)


ComplexREACT_27010 (Reactome)
MOCS3(Zn++) (ox.) ComplexREACT_26398 (Reactome)


ComplexREACT_25705 (Reactome)


ComplexREACT_11452 (Reactome)


ComplexREACT_11644 (Reactome)
Magnesium Metabolite18420 (ChEBI)
MoCo Metabolite25372 (ChEBI)
Molybdopterin Metabolite58698 (ChEBI)
NAD+ Metabolite15846 (ChEBI)
NAD+ kinase

homotetramer (Zn2+)

ComplexREACT_11972 (Reactome)
NADH Metabolite16908 (ChEBI)
NADP+ Metabolite18009 (ChEBI)
NADPH Metabolite16474 (ChEBI)


ComplexREACT_11254 (Reactome)
NFS1 holoenzyme ComplexREACT_26174 (Reactome)
NH3 Metabolite16134 (ChEBI)
NH4+ Metabolite28938 (ChEBI)


ComplexREACT_11954 (Reactome)


ComplexREACT_11885 (Reactome)


ComplexREACT_11927 (Reactome)


ProteinP43490 (UniProt)
Nicotinamide Metabolite17154 (ChEBI)


REACT_11492 (Reactome)
Nicotinate Metabolite15940 (ChEBI)

phosphoribosylt ransferase homodimer

ComplexREACT_17164 (Reactome)
Nicotinate D-


Metabolite15763 (ChEBI)

nucleotide pyrophosphoryl ase

ProteinQ15274 (UniProt)

pyrophosphatase homodimer

ComplexREACT_11259 (Reactome)


Metabolite18367 (ChEBI)


Metabolite18367 (ChEBI)
Orthophosphate Metabolite18367 (ChEBI)
Oxygen Metabolite15379 (ChEBI)
PANK1;3;4 ProteinREACT_11650 (Reactome)
PAP Metabolite17985 (ChEBI)
PDXK holoenzyme ComplexREACT_26520 (Reactome)


ComplexREACT_26517 (Reactome)

synthase homodimer

ComplexREACT_11296 (Reactome)

homotrimer (FMN cofactor)

ComplexREACT_11983 (Reactome)
PRPP Metabolite17111 (ChEBI)
Pantothenate Metabolite7916 (ChEBI)

kinase 2

ProteinQ9BZ23 (UniProt)


Metabolite15905 (ChEBI)


Metabolite15769 (ChEBI)
Precursor Z Metabolite59648 (ChEBI)
Pyridoxal Metabolite17310 (ChEBI)


Metabolite18405 (ChEBI)
Pyridoxamine Metabolite16410 (ChEBI)


Metabolite18335 (ChEBI)
Pyridoxine Metabolite16709 (ChEBI)


Metabolite28803 (ChEBI)
Quinolinate Metabolite46828 (ChEBI)
Riboflavin Metabolite17015 (ChEBI)

kinase (Mg2+)

ComplexREACT_11696 (Reactome)
S-adenosylmethionine Metabolite15414 (ChEBI)


ComplexREACT_3102 (Reactome)
SLC19A1 ProteinP41440 (UniProt)


ProteinREACT_11312 (Reactome)
SLC25A16 ProteinP16260 (UniProt)
SLC25A32 ProteinQ9H2D1 (UniProt)
SLC46A1 ProteinQ96NT5 (UniProt)
SMVT ProteinQ9Y289 (UniProt)


ProteinREACT_11407 (Reactome)

polygluta mate

Metabolite28624 (ChEBI)
THF-polyglutamate Metabolite28624 (ChEBI)
THTPA-Mg++ ComplexREACT_26403 (Reactome)

homodimer (Mg2+)

ComplexREACT_11433 (Reactome)


ComplexREACT_11966 (Reactome)
Tetrahydrofolate Metabolite15635 (ChEBI)
ThDP kinase REACT_26649 (Reactome)
Thiamin Metabolite18385 (ChEBI)


Metabolite9532 (ChEBI)


Metabolite9534 (ChEBI)
ascorbate Metabolite29073 (ChEBI)
dehydroascorbate Metabolite17242 (ChEBI)


ComplexREACT_11821 (Reactome)


ComplexREACT_11661 (Reactome)
glycine Metabolite15428 (ChEBI)
molybdate(2-) Metabolite36264 (ChEBI)


Metabolite17858 (ChEBI)


Metabolite29888 (ChEBI)


Metabolite16504 (ChEBI)


Metabolite29101 (ChEBI)
sulfurated MoCo Metabolite60102 (ChEBI)

Annotated Interactions

No annotated interactions

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