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

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12, 422425, 5913, 44, 7012, 4253, 75771061, 635133, 5929, 41, 58, 6212, 4246, 5225, 5933, 5912, 4233, 5916, 7120, 69653, 7539, 541, 2, 31, 50, 60...34, 5148, 7315, 5798, 381846, 5223, 6526, 27, 40, 43, 636615, 5712, 4276473046, 52423, 6519, 3614285, 21, 64, 6714637, 5935222232, 5635511, 547429, 41, 45, 4917, 29, 41937, 59nucleoplasmcytosolGolgi lumenmitochondrial matrixPRPPNicotinamideADPNicotinate D-ribonucleotideATPCoA-SHNADHsulfurated MoCoGLUT1; GLUT3ATPsodium ionoxidized glutathioneATPPRPPRiboflavinH2OBiotinNFS1 holoenzymeH+MOCOS holoenzymeMOCS2-CO-S(1-) tetramerAMPL-GlutamateCO2Thiaminsodium ionNH4+ATPATPPDXK holoenzymeMTHFR dimerL-GlutamineATPpyrophosphatePrecursor ZATPNicotinateH2OascorbatePPCDC homotrimer (FMN cofactor)ATPL-GlutamatepyrophosphateH2ONicotinate D-ribonucleotideH+NH3DehydroascorbateH+TPK1 homodimer (Mg2+)NAD+5-methyltetrahydrofolate polyglutamatePPC synthase homodimerNicotinamide deaminaseMoCoPantothenate kinase 2MOCS3(Zn++) (ox.)PNPO holoenzymeferric CYB5AADPFatty acid synthase apoenzymeFAD1 flavin adenine dinucleotide synthetase homologCoA-SHpyrophosphateFolylpolyglutamate synthase-1AMPpyrophosphateL-cysteineAASDHPPTsodium ionH2OglycineH2OPyridoxalATPS-adenosylmethionineTetrahydrofolateH2OSVCT1; SVCT2Riboflavin kinase (Mg2+)Gephyrin(Mg++) trimer5-methyltetrahydrofolateQuinolinateATPPRPPH2OADPNicotinate D-ribonucleotideCYB5R3:FADNicotinate phosphoribosyltransferase homodimerMOCS2 tetramerATPGTPH2OTHTPA-Mg++MolybdopterinADPOrthophosphateFolateTHF-polyglutamateAscorbateOrthophosphateOrthophosphateADPADPATPPantothenateATPBifunctional coenzyme A synthasePantothenateNADP+ADPOxygenpyrophosphateADPAMPDihydrofolatePhosphopantothenoylcysteinePyridoxinepyrophosphateNADPHPANK1;3;45-methyltetrahydrofolateFatty acid synthasePyridoxal phosphateNADPHSLC19A1ThDP kinaseFormateNADPHSLC46A1Pyridoxamine phosphateGlutathioneNMNAT2 (Mg2+)MOCS1 holoenzymeFADNADSYN1 homohexamerH+H+OrthophosphateNADP+ATPThiamin triphosphatepyrophosphateSLC19A2; SLC19A32-Amino-3-carboxymuconate semialdehydeGSTO homodimersMOCS2A-CO-S(1-)NADP+SLC25A16SLC25A32Nucleotide pyrophosphatase homodimerNicotinate-nucleotide pyrophosphorylaseNicotinate D-ribonucleotidesodium ion5,10-methenyltetrahydrofolate polyglutamatepyrophosphate10-formyltetrahydrofolate polyglutamateferrous CYB5AH2OPyridoxamine5,10-methylenetetrahydrofolate polyglutamateH+pyrophosphate4' phosphopantetheineNMNAT3 tetramerL-GlutamatepyrophosphateL-Alanine5'-DeoxyadenosineSMVTMTHFD1 dimerNMNAT1 hexamerL-serineFolateL-AlanineATPDeamino-NAD+H+ATPdehydroascorbateTRAP homodimerH+NamPRTTHF-polyglutamateMOCS3(Zn++)-S-S(1-)Flavin mononucleotideTetrahydrofolateATPADPThiamin diphosphateADPCO2pyrophosphateNAD+ kinase homotetramer (Zn2+)pyrophosphateAMPDihydrofolate reductaseNAD+Dephospho-CoAPhospho-pantothenateADPAMPmolybdate(2-)ATPMagnesiumPyridoxine phosphateL-cysteineH2OH2O2H2OOrthophosphateNADP+L-MethionineSHMT1 tetramerBiotinsemidehydroascorbateMOCS2APAPL-cysteineATPThiaminAMPFolylpolyglutamate synthase-2Deamino-NAD+MOCS3(Zn++) (red.)7


Description

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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  108. 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
  109. Schmuck EM, Board PG, Whitbread AK, Tetlow N, Cavanaugh JA, Blackburn AC, Masoumi A.; ''Characterization of the monomethylarsonate reductase and dehydroascorbate reductase activities of Omega class glutathione transferase variants: implications for arsenic metabolism and the age-at-onset of Alzheimer's and Parkinson's diseases.''; PubMed Europe PMC Scholia
  110. Froese DS, Kochan G, Muniz JR, Wu X, Gileadi C, Ugochukwu E, Krysztofinska E, Gravel RA, Oppermann U, Yue WW.; ''Structures of the human GTPase MMAA and vitamin B12-dependent methylmalonyl-CoA mutase and insight into their complex formation.''; PubMed Europe PMC Scholia
  111. Daugherty M, Polanuyer B, Farrell M, Scholle M, Lykidis A, de Crécy-Lagard V, Osterman A.; ''Complete reconstitution of the human coenzyme A biosynthetic pathway via comparative genomics.''; PubMed Europe PMC Scholia
  112. Zhou B, Westaway SK, Levinson B, Johnson MA, Gitschier J, Hayflick SJ.; ''A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome.''; PubMed Europe PMC Scholia
  113. Doolin MT, Barbaux S, McDonnell M, Hoess K, Whitehead AS, Mitchell LE.; ''Maternal genetic effects, exerted by genes involved in homocysteine remethylation, influence the risk of spina bifida.''; PubMed Europe PMC Scholia
  114. Yao Y, Yonezawa A, Yoshimatsu H, Masuda S, Katsura T, Inui K.; ''Identification and comparative functional characterization of a new human riboflavin transporter hRFT3 expressed in the brain.''; PubMed Europe PMC Scholia
  115. 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
  116. Yoshimatsu H, Yonezawa A, Yao Y, Sugano K, Nakagawa S, Omura T, Matsubara K.; ''Functional involvement of RFVT3/SLC52A3 in intestinal riboflavin absorption.''; PubMed Europe PMC Scholia
  117. Plesa M, Kim J, Paquette SG, Gagnon H, Ng-Thow-Hing C, Gibbs BF, Hancock MA, Rosenblatt DS, Coulton JW.; ''Interaction between MMACHC and MMADHC, two human proteins participating in intracellular vitamin B₁₂ metabolism.''; PubMed Europe PMC Scholia
  118. Randaccio L, Geremia S, Demitri N, Wuerges J.; ''Vitamin B12: unique metalorganic compounds and the most complex vitamins.''; PubMed Europe PMC Scholia
  119. Ramaswamy G, Karim MA, Murti KG, Jackowski S.; ''PPARalpha controls the intracellular coenzyme A concentration via regulation of PANK1alpha gene expression.''; PubMed Europe PMC Scholia
  120. 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
  121. Johnston J, Bollekens J, Allen RH, Berliner N.; ''Structure of the cDNA encoding transcobalamin I, a neutrophil granule protein.''; PubMed Europe PMC Scholia
  122. Wuerges J, Garau G, Geremia S, Fedosov SN, Petersen TE, Randaccio L.; ''Structural basis for mammalian vitamin B12 transport by transcobalamin.''; PubMed Europe PMC Scholia
  123. Nielsen MJ, Rasmussen MR, Andersen CB, Nexø E, Moestrup SK.; ''Vitamin B12 transport from food to the body's cells--a sophisticated, multistep pathway.''; PubMed Europe PMC Scholia
  124. Zhao R, Gao F, Goldman ID.; ''Molecular cloning of human thiamin pyrophosphokinase.''; PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
114645view16:11, 25 January 2021ReactomeTeamReactome version 75
113093view11:15, 2 November 2020ReactomeTeamReactome version 74
112327view15:25, 9 October 2020ReactomeTeamReactome version 73
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

DataNodes

View all...
NameTypeDatabase referenceComment
10-

formyltetrahydro folate polyglutamate

MetaboliteREACT_4874 (Reactome)
2-Amino-3-

carboxymuconate semialdehyde

Metabolite29044 (ChEBI)
4'

phosphopantet heine

Metabolite16858 (ChEBI)
5'-Deoxyadenosine Metabolite17319 (ChEBI)
5,10-

methenyltetra hydrofolate polyglutamate

MetaboliteREACT_11283 (Reactome)
5,10-

methylenetetrahyd rofolate polyglutamate

MetaboliteREACT_11343 (Reactome)
5-

methyltetrahydro folate

Metabolite15641 (ChEBI)
5-

methyltetrahydro folate polyglutamate

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

coenzyme A synthase

ProteinQ13057 (UniProt)
Biotin Metabolite15956 (ChEBI)
CO2 Metabolite16526 (ChEBI)
CYB5R3:

FAD

ComplexREACT_11367 (Reactome)
CoA-SH Metabolite15346 (ChEBI)
Deamino-

NAD+

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

reductase

ProteinP00374 (UniProt)
FAD Metabolite16238 (ChEBI)
FAD1 flavin

adenine dinucleotide synthetase homolog

ProteinQ8NFF5 (UniProt)
Fatty acid

synthase

ProteinP49327 (UniProt)
Fatty acid

synthase apoenzyme

ProteinP49327 (UniProt)
Flavin

mononucleoti de

Metabolite17621 (ChEBI)
Folate Metabolite27470 (ChEBI)
Folylpolyglut

amate synthase-2

ProteinQ05932-2 (UniProt)
Folylpolyglutamate

synthase-1

ProteinQ05932-1 (UniProt)
Formate Metabolite30751 (ChEBI)
GLUT1;

GLUT3

ProteinREACT_11567 (Reactome)
GSTO

homodimers

ComplexREACT_11879 (Reactome)
GTP Metabolite15996 (ChEBI)
Gephyrin(Mg++)

trimer

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)
MOCS1

holoenzyme

ComplexREACT_26979 (Reactome)
MOCS2

tetramer

ComplexREACT_25714 (Reactome)
MOCS2-CO-S(1-)

tetramer

ComplexREACT_25716 (Reactome)
MOCS2A ProteinO96033 (UniProt)
MOCS2A-CO-S(1-) ProteinO96033 (UniProt)
MOCS3(Zn++)

(red.)

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

(1-)

ComplexREACT_25705 (Reactome)
MTHFD1

dimer

ComplexREACT_11452 (Reactome)
MTHFR

dimer

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)
NADSYN1

homohexamer

ComplexREACT_11254 (Reactome)
NFS1 holoenzyme ComplexREACT_26174 (Reactome)
NH3 Metabolite16134 (ChEBI)
NH4+ Metabolite28938 (ChEBI)
NMNAT1

hexamer

ComplexREACT_11954 (Reactome)
NMNAT2

(Mg2+)

ComplexREACT_11885 (Reactome)
NMNAT3

tetramer

ComplexREACT_11927 (Reactome)
NamPR

T

ProteinP43490 (UniProt)
Nicotinamide Metabolite17154 (ChEBI)
Nicotinamide

deaminase

REACT_11492 (Reactome)
Nicotinate Metabolite15940 (ChEBI)
Nicotinate

phosphoribosylt ransferase homodimer

ComplexREACT_17164 (Reactome)
Nicotinate D-

ribonucleotide

Metabolite15763 (ChEBI)
Nicotinate-

nucleotide pyrophosphoryl ase

ProteinQ15274 (UniProt)
Nucleotide

pyrophosphatase homodimer

ComplexREACT_11259 (Reactome)
Orthophos

phate

Metabolite18367 (ChEBI)
Orthophosp

hate

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

holoenzyme

ComplexREACT_26517 (Reactome)
PPC

synthase homodimer

ComplexREACT_11296 (Reactome)
PPCDC

homotrimer (FMN cofactor)

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

kinase 2

ProteinQ9BZ23 (UniProt)
Phospho-

pantothenate

Metabolite15905 (ChEBI)
Phosphopantotheno

ylcysteine

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

phosphate

Metabolite18405 (ChEBI)
Pyridoxamine Metabolite16410 (ChEBI)
Pyridoxamine

phosphate

Metabolite18335 (ChEBI)
Pyridoxine Metabolite16709 (ChEBI)
Pyridoxine

phosphate

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

kinase (Mg2+)

ComplexREACT_11696 (Reactome)
S-adenosylmethionine Metabolite15414 (ChEBI)
SHMT1

tetramer

ComplexREACT_3102 (Reactome)
SLC19A1 ProteinP41440 (UniProt)
SLC19A2;

SLC19A3

ProteinREACT_11312 (Reactome)
SLC25A16 ProteinP16260 (UniProt)
SLC25A32 ProteinQ9H2D1 (UniProt)
SLC46A1 ProteinQ96NT5 (UniProt)
SMVT ProteinQ9Y289 (UniProt)
SVCT1;

SVCT2

ProteinREACT_11407 (Reactome)
THF-

polygluta mate

Metabolite28624 (ChEBI)
THF-polyglutamate Metabolite28624 (ChEBI)
THTPA-Mg++ ComplexREACT_26403 (Reactome)
TPK1

homodimer (Mg2+)

ComplexREACT_11433 (Reactome)
TRAP

homodimer

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

diphosphate

Metabolite9532 (ChEBI)
Thiamin

triphosphate

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

CYB5A

ComplexREACT_11821 (Reactome)
ferrous

CYB5A

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

glutathione

Metabolite17858 (ChEBI)
pyrophosp

hate

Metabolite29888 (ChEBI)
semidehydroa

scorbate

Metabolite16504 (ChEBI)
sodium

ion

Metabolite29101 (ChEBI)
sulfurated MoCo Metabolite60102 (ChEBI)

Annotated Interactions

No annotated interactions

Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP1857"
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