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

From WikiPathways

Revision as of 10:24, 6 October 2011 by MartijnVanIersel (Talk | contribs)
Jump to: navigation, search
48, 73746926, 27, 40, 43, 6333, 5933, 5939, 543553, 7529, 41, 45, 498, 381, 2, 31, 50, 60...53, 7515, 5746, 5212, 42761446, 5213, 44, 7012, 426617, 29, 4112, 4247934, 5119, 362237, 5925, 5928461, 6312, 425, 21, 64, 67312, 421023, 653020, 69511446, 5233, 591832, 562223, 6511, 547715, 5725, 592437, 5916, 7129, 41, 58, 62655mitochondrial matrixGolgi lumennucleoplasmcytosolMagnesiumH2OOrthophosphatepyrophosphateTetrahydrofolateATPferric CYB5AThDP kinasepyrophosphateDehydroascorbateADPGLUT1; GLUT3GlutathioneFolylpolyglutamate synthase-1QuinolinateTPK1 homodimer (Mg2+)FAD1 flavin adenine dinucleotide synthetase homologAASDHPPTL-AlaninePDXK holoenzymesulfurated MoCoAMPThiamin diphosphateATPpyrophosphateThiaminSMVTH2O2RiboflavinNMNAT1 hexamerPyridoxal phosphateSHMT1 tetramerH2OH+Nicotinate D-ribonucleotide5'-DeoxyadenosineL-GlutamateNicotinamide2-Amino-3-carboxymuconate semialdehydesodium ion4' phosphopantetheinesodium ionFolylpolyglutamate synthase-2Nicotinate D-ribonucleotideMOCS2-CO-S(1-) tetramerPRPPNADP+NicotinateNADPHAMPNADSYN1 homohexamerL-GlutamatePhosphopantothenoylcysteineNH3H2OADPMOCS2ANH4+ferrous CYB5AATPSLC25A16FADADPBifunctional coenzyme A synthaseTHF-polyglutamateNAD+AMPNFS1 holoenzymesodium ionMTHFD1 dimerL-serineNMNAT3 tetramerATPL-GlutaminePNPO holoenzyme5-methyltetrahydrofolate polyglutamateNucleotide pyrophosphatase homodimerAscorbateFlavin mononucleotideMoCo5-methyltetrahydrofolateH2OL-cysteineNamPRTNAD+ kinase homotetramer (Zn2+)OrthophosphatePRPPNAD+Nicotinate D-ribonucleotideMolybdopterinRiboflavin kinase (Mg2+)S-adenosylmethionineOxygenThiamin triphosphateH2OATPATPFolateADPsodium ionH2OFormateOrthophosphatePhospho-pantothenatePPCDC homotrimer (FMN cofactor)TRAP homodimerPANK1;3;4Dephospho-CoAPantothenateDihydrofolate reductasePyridoxinePyridoxine phosphateMOCS2A-CO-S(1-)NADP+Gephyrin(Mg++) trimerNADPHL-cysteineATPMOCS1 holoenzymeSLC19A1Pantothenate kinase 2SLC46A1PAPADPH+5-methyltetrahydrofolateADPATPH+FolatePyridoxamineCYB5R3:FADDeamino-NAD+Precursor ZpyrophosphateL-GlutamateNADP+NADHH2O5,10-methylenetetrahydrofolate polyglutamatepyrophosphateAMPNicotinate-nucleotide pyrophosphorylaseThiaminBiotinoxidized glutathioneMOCS3(Zn++) (ox.)MTHFR dimerCoA-SHTetrahydrofolateH2ONicotinate D-ribonucleotideATPascorbateH2OATPADPsemidehydroascorbatemolybdate(2-)H+5,10-methenyltetrahydrofolate polyglutamateMOCS2 tetramerPRPPCO2pyrophosphateSLC25A32NMNAT2 (Mg2+)MOCS3(Zn++)-S-S(1-)L-MethioninePantothenate10-formyltetrahydrofolate polyglutamateDeamino-NAD+NADPHpyrophosphateFatty acid synthaseL-cysteineSLC19A2; SLC19A3ATPNADP+pyrophosphateOrthophosphateH+Nicotinamide deaminasedehydroascorbateADPMOCS3(Zn++) (red.)ADPH+Pyridoxamine phosphateATPATPPyridoxalpyrophosphateAMPADPAMPPPC synthase homodimerDihydrofolateH+ATPATPCoA-SHpyrophosphateATPCO2Nicotinate phosphoribosyltransferase homodimerADPMOCOS holoenzymeGTPH2OL-AlanineTHF-polyglutamateATPH2OglycineBiotinSVCT1; SVCT2ATPFatty acid synthase apoenzymeATPTHTPA-Mg++GSTO homodimersH+Orthophosphatepyrophosphatepyrophosphate7


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>

Try the New WikiPathways

View approved pathways at the new wikipathways.org.

Quality Tags

Ontology Terms

 

Bibliography

View all...
  1. Board PG, Coggan M, Chelvanayagam G, Easteal S, Jermiin LS, Schulte GK, Danley DE, Hoth LR, Griffor MC, Kamath AV, Rosner MH, Chrunyk BA, Perregaux DE, Gabel CA, Geoghegan KF, Pandit J.; ''Identification, characterization, and crystal structure of the Omega class glutathione transferases.''; PubMed Europe PMC Scholia
  2. Quadros EV, Rothenberg SP, Pan YC, Stein S.; ''Purification and molecular characterization of human transcobalamin II.''; PubMed Europe PMC Scholia
  3. Goding JW, Grobben B, Slegers H.; ''Physiological and pathophysiological functions of the ecto-nucleotide pyrophosphatase/phosphodiesterase family.''; PubMed Europe PMC Scholia
  4. Kim J, Gherasim C, Banerjee R.; ''Decyanation of vitamin B12 by a trafficking chaperone.''; PubMed Europe PMC Scholia
  5. Du J, Cullen JJ, Buettner GR.; ''Ascorbic acid: chemistry, biology and the treatment of cancer.''; PubMed Europe PMC Scholia
  6. 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
  7. Maras B, Barra D, Duprè S, Pitari G.; ''Is pantetheinase the actual identity of mouse and human vanin-1 proteins?''; PubMed Europe PMC Scholia
  8. Johnson MA, Kuo YM, Westaway SK, Parker SM, Ching KH, Gitschier J, Hayflick SJ.; ''Mitochondrial localization of human PANK2 and hypotheses of secondary iron accumulation in pantothenate kinase-associated neurodegeneration.''; PubMed Europe PMC Scholia
  9. Hänzelmann P, Schwarz G, Mendel RR.; ''Functionality of alternative splice forms of the first enzymes involved in human molybdenum cofactor biosynthesis.''; PubMed Europe PMC Scholia
  10. Hartmann T, Terao M, Garattini E, Teutloff C, Alfaro JF, Jones JP, Leimkühler S.; ''The impact of single nucleotide polymorphisms on human aldehyde oxidase.''; PubMed Europe PMC Scholia
  11. Labay V, Raz T, Baron D, Mandel H, Williams H, Barrett T, Szargel R, McDonald L, Shalata A, Nosaka K, Gregory S, Cohen N.; ''Mutations in SLC19A2 cause thiamine-responsive megaloblastic anaemia associated with diabetes mellitus and deafness.''; PubMed Europe PMC Scholia
  12. Wang H, Huang W, Fei YJ, Xia H, Yang-Feng TL, Leibach FH, Devoe LD, Ganapathy V, Prasad PD.; ''Human placental Na+-dependent multivitamin transporter. Cloning, functional expression, gene structure, and chromosomal localization.''; PubMed Europe PMC Scholia
  13. 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
  14. Hannibal L, Kim J, Brasch NE, Wang S, Rosenblatt DS, Banerjee R, Jacobsen DW.; ''Processing of alkylcobalamins in mammalian cells: A role for the MMACHC (cblC) gene product.''; PubMed Europe PMC Scholia
  15. Xiang S, Tong L.; ''Crystal structures of human and Staphylococcus aureus pyruvate carboxylase and molecular insights into the carboxyltransfer reaction.''; PubMed Europe PMC Scholia
  16. Rajan DP, Huang W, Dutta B, Devoe LD, Leibach FH, Ganapathy V, Prasad PD.; ''Human placental sodium-dependent vitamin C transporter (SVCT2): molecular cloning and transport function.''; PubMed Europe PMC Scholia
  17. Mah W, Deme JC, Watkins D, Fung S, Janer A, Shoubridge EA, Rosenblatt DS, Coulton JW.; ''Subcellular location of MMACHC and MMADHC, two human proteins central to intracellular vitamin B(12) metabolism.''; PubMed Europe PMC Scholia
  18. 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
  19. Shah NP, Beech CM, Sturm AC, Tanner SM.; ''Investigation of the ABC transporter MRP1 in selected patients with presumed defects in vitamin B12 absorption.''; PubMed Europe PMC Scholia
  20. Fleming JC, Tartaglini E, Steinkamp MP, Schorderet DF, Cohen N, Neufeld EJ.; ''The gene mutated in thiamine-responsive anaemia with diabetes and deafness (TRMA) encodes a functional thiamine transporter.''; PubMed Europe PMC Scholia
  21. Wilson A, Leclerc D, Rosenblatt DS, Gravel RA.; ''Molecular basis for methionine synthase reductase deficiency in patients belonging to the cblE complementation group of disorders in folate/cobalamin metabolism.''; PubMed Europe PMC Scholia
  22. Subramanian VS, Marchant JS, Said HM.; ''Targeting and trafficking of the human thiamine transporter-2 in epithelial cells.''; PubMed Europe PMC Scholia
  23. Hayman AR, Warburton MJ, Pringle JA, Coles B, Chambers TJ.; ''Purification and characterization of a tartrate-resistant acid phosphatase from human osteoclastomas.''; PubMed Europe PMC Scholia
  24. Chandler CS, Ballard FJ.; ''Distribution and degradation of biotin-containing carboxylases in human cell lines.''; PubMed Europe PMC Scholia
  25. Takahashi-Íñiguez T, García-Arellano H, Trujillo-Roldán MA, Flores ME.; ''Protection and reactivation of human methylmalonyl-CoA mutase by MMAA protein.''; PubMed Europe PMC Scholia
  26. Ingaramo M, Beckett D.; ''Selectivity in post-translational biotin addition to five human carboxylases.''; PubMed Europe PMC Scholia
  27. Siu VM, Ratko S, Prasad AN, Prasad C, Rupar CA.; ''Amish microcephaly: Long-term survival and biochemical characterization.''; PubMed Europe PMC Scholia
  28. Leal NA, Park SD, Kima PE, Bobik TA.; ''Identification of the human and bovine ATP:Cob(I)alamin adenosyltransferase cDNAs based on complementation of a bacterial mutant.''; PubMed Europe PMC Scholia
  29. Brizio C, Galluccio M, Wait R, Torchetti EM, Bafunno V, Accardi R, Gianazza E, Indiveri C, Barile M.; ''Over-expression in Escherichia coli and characterization of two recombinant isoforms of human FAD synthetase.''; PubMed Europe PMC Scholia
  30. Aminoff M, Carter JE, Chadwick RB, Johnson C, Gräsbeck R, Abdelaal MA, Broch H, Jenner LB, Verroust PJ, Moestrup SK, de la Chapelle A, Krahe R.; ''Mutations in CUBN, encoding the intrinsic factor-vitamin B12 receptor, cubilin, cause hereditary megaloblastic anaemia 1.''; PubMed Europe PMC Scholia
  31. Leclerc D, Campeau E, Goyette P, Adjalla CE, Christensen B, Ross M, Eydoux P, Rosenblatt DS, Rozen R, Gravel RA.; ''Human methionine synthase: cDNA cloning and identification of mutations in patients of the cblG complementation group of folate/cobalamin disorders.''; PubMed Europe PMC Scholia
  32. Wolf B, Jensen K.; ''Evolutionary conservation of biotinidase: implications for the enzyme's structure and subcellular localization.''; PubMed Europe PMC Scholia
  33. Leonardi R, Rock CO, Jackowski S, Zhang YM.; ''Activation of human mitochondrial pantothenate kinase 2 by palmitoylcarnitine.''; PubMed Europe PMC Scholia
  34. Gailus S, Höhne W, Gasnier B, Nürnberg P, Fowler B, Rutsch F.; ''Insights into lysosomal cobalamin trafficking: lessons learned from cblF disease.''; PubMed Europe PMC Scholia
  35. Batra J, Szabó A, Caulfield TR, Soares AS, Sahin-Tóth M, Radisky ES.; ''Long-range electrostatic complementarity governs substrate recognition by human chymotrypsin C, a key regulator of digestive enzyme activation.''; PubMed Europe PMC Scholia
  36. Srikumar K, Premalatha R.; ''Effect of gastrointestinal proteases on purified human intrinsic factor-vitamin B12 (IF-B12) complex.''; PubMed Europe PMC Scholia
  37. Prasad PD, Wang H, Huang W, Fei YJ, Leibach FH, Devoe LD, Ganapathy V.; ''Molecular and functional characterization of the intestinal Na+-dependent multivitamin transporter.''; PubMed Europe PMC Scholia
  38. Joshi AK, Zhang L, Rangan VS, Smith S.; ''Cloning, expression, and characterization of a human 4'-phosphopantetheinyl transferase with broad substrate specificity.''; PubMed Europe PMC Scholia
  39. Wolthers KR, Lou X, Toogood HS, Leys D, Scrutton NS.; ''Mechanism of coenzyme binding to human methionine synthase reductase revealed through the crystal structure of the FNR-like module and isothermal titration calorimetry.''; PubMed Europe PMC Scholia
  40. Belli SI, Goding JW.; ''Biochemical characterization of human PC-1, an enzyme possessing alkaline phosphodiesterase I and nucleotide pyrophosphatase activities.''; PubMed Europe PMC Scholia
  41. Nabokina SM, Subramanian VS, Said HM.; ''Association of PDZ-containing protein PDZD11 with the human sodium-dependent multivitamin transporter.''; PubMed Europe PMC Scholia
  42. Strauss E, Zhai H, Brand LA, McLafferty FW, Begley TP.; ''Mechanistic studies on phosphopantothenoylcysteine decarboxylase: trapping of an enethiolate intermediate with a mechanism-based inactivating agent.''; PubMed Europe PMC Scholia
  43. Yonezawa A, Masuda S, Katsura T, Inui K.; ''Identification and functional characterization of a novel human and rat riboflavin transporter, RFT1.''; PubMed Europe PMC Scholia
  44. Hänzelmann P, Hernández HL, Menzel C, García-Serres R, Huynh BH, Johnson MK, Mendel RR, Schindelin H.; ''Characterization of MOCS1A, an oxygen-sensitive iron-sulfur protein involved in human molybdenum cofactor biosynthesis.''; PubMed Europe PMC Scholia
  45. Kang J, Samuels DC.; ''The evidence that the DNC (SLC25A19) is not the mitochondrial deoxyribonucleotide carrier.''; PubMed Europe PMC Scholia
  46. 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
  47. Wolthers KR, Scrutton NS.; ''Protein interactions in the human methionine synthase-methionine synthase reductase complex and implications for the mechanism of enzyme reactivation.''; PubMed Europe PMC Scholia
  48. Stucki M, Coelho D, Suormala T, Burda P, Fowler B, Baumgartner MR.; ''Molecular mechanisms leading to three different phenotypes in the cblD defect of intracellular cobalamin metabolism.''; PubMed Europe PMC Scholia
  49. Lee HS, Moon BJ, Choi SY, Kwon OS.; ''Human pyridoxal kinase: overexpression and properties of the recombinant enzyme.''; PubMed Europe PMC Scholia
  50. Nosaka K, Onozuka M, Kakazu N, Hibi S, Nishimura H, Nishino H, Abe T.; ''Isolation and characterization of a human thiamine pyrophosphokinase cDNA.''; PubMed Europe PMC Scholia
  51. Quadros EV.; ''Advances in the understanding of cobalamin assimilation and metabolism.''; PubMed Europe PMC Scholia
  52. 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
  53. Abu-Elheiga L, Brinkley WR, Zhong L, Chirala SS, Woldegiorgis G, Wakil SJ.; ''The subcellular localization of acetyl-CoA carboxylase 2.''; PubMed Europe PMC Scholia
  54. 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
  55. 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
  56. 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
  57. Wuebbens MM, Rajagopalan KV.; ''Mechanistic and mutational studies of Escherichia coli molybdopterin synthase clarify the final step of molybdopterin biosynthesis.''; PubMed Europe PMC Scholia
  58. Wilson A, Platt R, Wu Q, Leclerc D, Christensen B, Yang H, Gravel RA, Rozen R.; ''A common variant in methionine synthase reductase combined with low cobalamin (vitamin B12) increases risk for spina bifida.''; PubMed Europe PMC Scholia
  59. Hiratsuka M, Sakamoto O, Li X, Suzuki Y, Aoki Y, Narisawa K.; ''Identification of holocarboxylase synthetase (HCS) proteins in human placenta.''; PubMed Europe PMC Scholia
  60. Deme JC, Miousse IR, Plesa M, Kim JC, Hancock MA, Mah W, Rosenblatt DS, Coulton JW.; ''Structural features of recombinant MMADHC isoforms and their interactions with MMACHC, proteins of mammalian vitamin B12 metabolism.''; PubMed Europe PMC Scholia
  61. Simpson GL, Ortwerth BJ.; ''The non-oxidative degradation of ascorbic acid at physiological conditions.''; PubMed Europe PMC Scholia
  62. Shirabe K, Landi MT, Takeshita M, Uziel G, Fedrizzi E, Borgese N.; ''A novel point mutation in a 3' splice site of the NADH-cytochrome b5 reductase gene results in immunologically undetectable enzyme and impaired NADH-dependent ascorbate regeneration in cultured fibroblasts of a patient with type II hereditary methemoglobinemia.''; PubMed Europe PMC Scholia
  63. Hymes J, Wolf B.; ''Human biotinidase isn't just for recycling biotin.''; PubMed Europe PMC Scholia
  64. Wang H, Dutta B, Huang W, Devoe LD, Leibach FH, Ganapathy V, Prasad PD.; ''Human Na(+)-dependent vitamin C transporter 1 (hSVCT1): primary structure, functional characteristics and evidence for a non-functional splice variant.''; PubMed Europe PMC Scholia
  65. Nishino K, Itokawa Y, Nishino N, Piros K, Cooper JR.; ''Enzyme system involved in the synthesis of thiamin triphosphate. I. Purification and characterization of protein-bound thiamin diphosphate: ATP phosphoryltransferase.''; PubMed Europe PMC Scholia
  66. Jansen R, Kalousek F, Fenton WA, Rosenberg LE, Ledley FD.; ''Cloning of full-length methylmalonyl-CoA mutase from a cDNA library using the polymerase chain reaction.''; PubMed Europe PMC Scholia
  67. Quadros EV, Nakayama Y, Sequeira JM.; ''The binding properties of the human receptor for the cellular uptake of vitamin B12.''; PubMed Europe PMC Scholia
  68. Marelja Z, Stöcklein W, Nimtz M, Leimkühler S.; ''A novel role for human Nfs1 in the cytoplasm: Nfs1 acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis.''; PubMed Europe PMC Scholia
  69. Aghajanian S, Worrall DM.; ''Identification and characterization of the gene encoding the human phosphopantetheine adenylyltransferase and dephospho-CoA kinase bifunctional enzyme (CoA synthase).''; PubMed Europe PMC Scholia
  70. Zhyvoloup A, Nemazanyy I, Panasyuk G, Valovka T, Fenton T, Rebholz H, Wang ML, Foxon R, Lyzogubov V, Usenko V, Kyyamova R, Gorbenko O, Matsuka G, Filonenko V, Gout IT.; ''Subcellular localization and regulation of coenzyme A synthase.''; PubMed Europe PMC Scholia
  71. 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
  72. Suzuki Y, Yang X, Aoki Y, Kure S, Matsubara Y.; ''Mutations in the holocarboxylase synthetase gene HLCS.''; PubMed Europe PMC Scholia
  73. Lakaye B, Makarchikov AF, Antunes AF, Zorzi W, Coumans B, De Pauw E, Wins P, Grisar T, Bettendorff L.; ''Molecular characterization of a specific thiamine triphosphatase widely expressed in mammalian tissues.''; PubMed Europe PMC Scholia
  74. 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
  75. 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
  76. Prohl C, Pelzer W, Diekert K, Kmita H, Bedekovics T, Kispal G, Lill R.; ''The yeast mitochondrial carrier Leu5p and its human homologue Graves' disease protein are required for accumulation of coenzyme A in the matrix.''; PubMed Europe PMC Scholia
  77. Lerner-Ellis JP, Tirone JC, Pawelek PD, Doré C, Atkinson JL, Watkins D, Morel CF, Fujiwara TM, Moras E, Hosack AR, Dunbar GV, Antonicka H, Forgetta V, Dobson CM, Leclerc D, Gravel RA, Shoubridge EA, Coulton JW, Lepage P, Rommens JM, Morgan K, Rosenblatt DS.; ''Identification of the gene responsible for methylmalonic aciduria and homocystinuria, cblC type.''; PubMed Europe PMC Scholia
  78. Quadros EV, Nakayama Y, Sequeira JM.; ''The protein and the gene encoding the receptor for the cellular uptake of transcobalamin-bound cobalamin.''; PubMed Europe PMC Scholia
  79. Mathews FS, Gordon MM, Chen Z, Rajashankar KR, Ealick SE, Alpers DH, Sukumar N.; ''Crystal structure of human intrinsic factor: cobalamin complex at 2.6-A resolution.''; PubMed Europe PMC Scholia
  80. Weatherly SC, Volrath SL, Elich TD.; ''Expression and characterization of recombinant fungal acetyl-CoA carboxylase and isolation of a soraphen-binding domain.''; PubMed Europe PMC Scholia
  81. Hörtnagel K, Prokisch H, Meitinger T.; ''An isoform of hPANK2, deficient in pantothenate kinase-associated neurodegeneration, localizes to mitochondria.''; PubMed Europe PMC Scholia
  82. Fan C, Bobik TA.; ''Functional characterization and mutation analysis of human ATP:Cob(I)alamin adenosyltransferase.''; PubMed Europe PMC Scholia
  83. Andersen CB, Madsen M, Storm T, Moestrup SK, Andersen GR.; ''Structural basis for receptor recognition of vitamin-B(12)-intrinsic factor complexes.''; PubMed Europe PMC Scholia
  84. Zhang YM, Rock CO, Jackowski S.; ''Biochemical properties of human pantothenate kinase 2 isoforms and mutations linked to pantothenate kinase-associated neurodegeneration.''; PubMed Europe PMC Scholia
  85. di Salvo ML, Hunt S, Schirch V.; ''Expression, purification, and kinetic constants for human and Escherichia coli pyridoxal kinases.''; PubMed Europe PMC Scholia
  86. Coelho D, Suormala T, Stucki M, Lerner-Ellis JP, Rosenblatt DS, Newbold RF, Baumgartner MR, Fowler B.; ''Gene identification for the cblD defect of vitamin B12 metabolism.''; PubMed Europe PMC Scholia
  87. Hymes J, Wolf B.; ''Biotinidase and its roles in biotin metabolism.''; PubMed Europe PMC Scholia
  88. Bailey LM, Wallace JC, Polyak SW.; ''Holocarboxylase synthetase: correlation of protein localisation with biological function.''; PubMed Europe PMC Scholia
  89. 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
  90. Dobson CM, Wai T, Leclerc D, Wilson A, Wu X, Doré C, Hudson T, Rosenblatt DS, Gravel RA.; ''Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements.''; PubMed Europe PMC Scholia
  91. Kang JH, Hong ML, Kim DW, Park J, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS.; ''Genomic organization, tissue distribution and deletion mutation of human pyridoxine 5'-phosphate oxidase.''; PubMed Europe PMC Scholia
  92. Tanner SM, Aminoff M, Wright FA, Liyanarachchi S, Kuronen M, Saarinen A, Massika O, Mandel H, Broch H, de la Chapelle A.; ''Amnionless, essential for mouse gastrulation, is mutated in recessive hereditary megaloblastic anemia.''; PubMed Europe PMC Scholia
  93. Rutsch F, Gailus S, Miousse IR, Suormala T, Sagné C, Toliat MR, Nürnberg G, Wittkampf T, Buers I, Sharifi A, Stucki M, Becker C, Baumgartner M, Robenek H, Marquardt T, Höhne W, Gasnier B, Rosenblatt DS, Fowler B, Nürnberg P.; ''Identification of a putative lysosomal cobalamin exporter altered in the cblF defect of vitamin B12 metabolism.''; PubMed Europe PMC Scholia
  94. Subramanian VS, Marchant JS, Said HM.; ''Biotin-responsive basal ganglia disease-linked mutations inhibit thiamine transport via hTHTR2: biotin is not a substrate for hTHTR2.''; PubMed Europe PMC Scholia
  95. 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
  96. Densupsoontorn N, Sanpakit K, Vijarnsorn C, Pattaragarn A, Kangwanpornsiri C, Jatutipsompol C, Tirapongporn H, Jirapinyo P, Shah NP, Sturm AC, Tanner SM.; ''Imerslund-Gräsbeck syndrome: new mutation in amnionless.''; PubMed Europe PMC Scholia
  97. Manoj N, Strauss E, Begley TP, Ealick SE.; ''Structure of human phosphopantothenoylcysteine synthetase at 2.3 A resolution.''; PubMed Europe PMC Scholia
  98. Daruwala R, Song J, Koh WS, Rumsey SC, Levine M.; ''Cloning and functional characterization of the human sodium-dependent vitamin C transporters hSVCT1 and hSVCT2.''; PubMed Europe PMC Scholia
  99. Borgese N, D'Arrigo A, De Silvestris M, Pietrini G.; ''NADH-cytochrome b5 reductase and cytochrome b5 isoforms as models for the study of post-translational targeting to the endoplasmic reticulum.''; PubMed Europe PMC Scholia
  100. Balamurugan K, Ortiz A, Said HM.; ''Biotin uptake by human intestinal and liver epithelial cells: role of the SMVT system.''; PubMed Europe PMC Scholia
  101. Liang WJ, Johnson D, Jarvis SM.; ''Vitamin C transport systems of mammalian cells.''; PubMed Europe PMC Scholia
  102. Youngdahl-Turner P, Mellman IS, Allen RH, Rosenberg LE.; ''Protein mediated vitamin uptake. Adsorptive endocytosis of the transcobalamin II-cobalamin complex by cultured human fibroblasts.''; PubMed Europe PMC Scholia
  103. Leimkuhler S, Freuer A, Araujo JA, Rajagopalan KV, Mendel RR.; ''Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency.''; PubMed Europe PMC Scholia
  104. Padovani D, Banerjee R.; ''Assembly and protection of the radical enzyme, methylmalonyl-CoA mutase, by its chaperone.''; PubMed Europe PMC Scholia
  105. 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
  106. Worgan LC, Niles K, Tirone JC, Hofmann A, Verner A, Sammak A, Kucic T, Lepage P, Rosenblatt DS.; ''Spectrum of mutations in mut methylmalonic acidemia and identification of a common Hispanic mutation and haplotype.''; PubMed Europe PMC Scholia
  107. Lerner-Ellis JP, Dobson CM, Wai T, Watkins D, Tirone JC, Leclerc D, Doré C, Lepage P, Gravel RA, Rosenblatt DS.; ''Mutations in the MMAA gene in patients with the cblA disorder of vitamin B12 metabolism.''; PubMed Europe PMC Scholia
  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"
Personal tools