1. Alanine--glyoxylate aminotransferase 2, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9BYV1 
Gene: AGXT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Lee IS, Muragaki Y, Ideguchi T, Hase T, Tsuji M, Ooshima A, Okuno E, Kido R: Molecular cloning and sequencing of a cDNA encoding alanine-glyoxylate aminotransferase 2 from rat kidney. J Biochem (Tokyo). 1995 Apr;117(4):856-62. Pubmed
- Takada Y, Mori T, Noguchi T: The effect of vitamin B6 deficiency on alanine: glyoxylate aminotransferase isoenzymes in rat liver. Arch Biochem Biophys. 1984 Feb 15;229(1):1-6. Pubmed
2. Glutamate decarboxylase 1
Pharmacological action: unknown
Actions: cofactor
Catalyzes the production of GABA
Organism class: human
UniProt ID: Q99259 
Gene: GAD1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Hwang IK, Yoo KY, Kim DS, Eum WS, Park JK, Park J, Kwon OS, Kang TC, Choi SY, Won MH: Changes of pyridoxal kinase expression and activity in the gerbil hippocampus following transient forebrain ischemia. Neuroscience. 2004;128(3):511-8. Pubmed
- Rust E, Martin DL, Chen CH: Cofactor and tryptophan accessibility and unfolding of brain glutamate decarboxylase. Arch Biochem Biophys. 2001 Aug 15;392(2):333-40. Pubmed
- Jin H, Sha D, Wei J, Davis KM, Wu H, Jin Y, Wu JY: Effect of apocalmodulin on recombinant human brain glutamic acid decarboxylase. J Neurochem. 2005 Feb;92(4):739-48. Pubmed
- Chen CH, Battaglioli G, Martin DL, Hobart SA, Colon W: Distinctive interactions in the holoenzyme formation for two isoforms of glutamate decarboxylase. Biochim Biophys Acta. 2003 Jan 31;1645(1):63-71. Pubmed
- Tong JC, Mackay IR, Chin J, Law RH, Fayad K, Rowley MJ: Enzymatic characterization of a recombinant isoform hybrid of glutamic acid decarboxylase (rGAD67/65) expressed in yeast. J Biotechnol. 2002 Aug 7;97(2):183-90. Pubmed
3. Cystathionine beta-synthase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P35520 
Gene: CBS 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Taoka S, Banerjee R: Stopped-flow kinetic analysis of the reaction catalyzed by the full-length yeast cystathionine beta-synthase. J Biol Chem. 2002 Jun 21;277(25):22421-5. Epub 2002 Apr 10. Pubmed
- Taoka S, Lepore BW, Kabil O, Ojha S, Ringe D, Banerjee R: Human cystathionine beta-synthase is a heme sensor protein. Evidence that the redox sensor is heme and not the vicinal cysteines in the CXXC motif seen in the crystal structure of the truncated enzyme. Biochemistry. 2002 Aug 20;41(33):10454-61. Pubmed
- Mino K, Ishikawa K: Characterization of a novel thermostable O-acetylserine sulfhydrylase from Aeropyrum pernix K1. J Bacteriol. 2003 Apr;185(7):2277-84. Pubmed
- Evande R, Ojha S, Banerjee R: Visualization of PLP-bound intermediates in hemeless variants of human cystathionine beta-synthase: evidence that lysine 119 is a general base. Arch Biochem Biophys. 2004 Jul 15;427(2):188-96. Pubmed
- Linnebank M, Janosik M, Kozich V, Pronicka E, Kubalska J, Sokolova J, Linnebank A, Schmidt E, Leyendecker C, Klockgether T, Kraus JP, Koch HG: The cystathionine beta-synthase (CBS) mutation c.1224-2A>C in Central Europe: Vitamin B6 nonresponsiveness and a common ancestral haplotype. Hum Mutat. 2004 Oct;24(4):352-3. Pubmed
4. Kynureninase
Pharmacological action: unknown
Actions: cofactor
Catalyzes the cleavage of L-kynurenine (L-Kyn) and L-3- hydroxykynurenine (L-3OHKyn) into anthranilic (AA) and 3- hydroxyanthranilic acids (3-OHAA), respectively. Has a preference for the L-3-hydroxy form. Also has cysteine-conjugate-beta-lyase activity
Organism class: human
UniProt ID: Q16719 
Gene: KYNU 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Momany C, Levdikov V, Blagova L, Lima S, Phillips RS: Three-dimensional structure of kynureninase from Pseudomonas fluorescens. Biochemistry. 2004 Feb 10;43(5):1193-203. Pubmed
- Rooseboom M, Vermeulen NP, Groot EJ, Commandeur JN: Tissue distribution of cytosolic beta-elimination reactions of selenocysteine Se-conjugates in rat and human. Chem Biol Interact. 2002 Aug 15;140(3):243-64. Pubmed
- Lima S, Khristoforov R, Momany C, Phillips RS: Crystal structure of Homo sapiens kynureninase. Biochemistry. 2007 Mar 13;46(10):2735-44. Epub 2007 Feb 15. Pubmed
- Walsh HA, Botting NP: Purification and biochemical characterization of some of the properties of recombinant human kynureninase. Eur J Biochem. 2002 Apr;269(8):2069-74. Pubmed
5. Serine hydroxymethyltransferase, cytosolic
Pharmacological action: unknown
Actions: cofactor
Interconversion of serine and glycine
Organism class: human
UniProt ID: P34896 
Gene: SHMT1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Trakatellis A, Dimitriadou A, Exindari M, Christodoulou D, Malissiovas N, Antoniadis A, Haitoglou K: Effect of combination of deoxypyridoxine with known anti-proliferative or immunosuppressive agents on lymphocyte serine hydroxymethyltransferase. Postgrad Med J. 1994;70 Suppl 1:S89-92. Pubmed
- Jagath JR, Sharma B, Rao NA, Savithri HS: The role of His-134, -147, and -150 residues in subunit assembly, cofactor binding, and catalysis of sheep liver cytosolic serine hydroxymethyltransferase. J Biol Chem. 1997 Sep 26;272(39):24355-62. Pubmed
- Bourguignon J, Neuburger M, Douce R: Resolution and characterization of the glycine-cleavage reaction in pea leaf mitochondria. Properties of the forward reaction catalysed by glycine decarboxylase and serine hydroxymethyltransferase. Biochem J. 1988 Oct 1;255(1):169-78. Pubmed
- Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed
6. Cysteine desulfurase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron- sulfur (Fe-S) clusters
Organism class: human
UniProt ID: Q9Y697 
Gene: NFS1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Ollagnier-De-Choudens S, Mulliez E, Hewitson KS, Fontecave M: Biotin synthase is a pyridoxal phosphate-dependent cysteine desulfurase. Biochemistry. 2002 Jul 23;41(29):9145-52. Pubmed
- You D, Wang L, Yao F, Zhou X, Deng Z: A novel DNA modification by sulfur: DndA is a NifS-like cysteine desulfurase capable of assembling DndC as an iron-sulfur cluster protein in Streptomyces lividans. Biochemistry. 2007 May 22;46(20):6126-33. Epub 2007 May 1. Pubmed
- Forlani F, Cereda A, Freuer A, Nimtz M, Leimkuhler S, Pagani S: The cysteine-desulfurase IscS promotes the production of the rhodanese RhdA in the persulfurated form. FEBS Lett. 2005 Dec 19;579(30):6786-90. Epub 2005 Nov 21. Pubmed
- Cupp-Vickery JR, Urbina H, Vickery LE: Crystal structure of IscS, a cysteine desulfurase from Escherichia coli. J Mol Biol. 2003 Jul 25;330(5):1049-59. Pubmed
- Frazzon J, Fick JR, Dean DR: Biosynthesis of iron-sulphur clusters is a complex and highly conserved process. Biochem Soc Trans. 2002 Aug;30(4):680-5. Pubmed
7. Aspartate aminotransferase, cytoplasmic
Pharmacological action: unknown
Actions: activator
Organism class: human
UniProt ID: P17174 
Gene: GOT1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Hansen CM, Shultz TD: Stability of vitamin B-6-dependent aminotransferase activity in frozen packed erythrocytes is dependent on storage temperature. J Nutr. 2001 May;131(5):1581-3. Pubmed
- Eliot AC, Kirsch JF: Modulation of the internal aldimine pK(a)’s of 1-aminocyclopropane-1-carboxylate synthase and aspartate aminotransferase by specific active site residues. Biochemistry. 2002 Mar 19;41(11):3836-42. Pubmed
- Cooper AJ, Bruschi SA, Anders MW: Toxic, halogenated cysteine S-conjugates and targeting of mitochondrial enzymes of energy metabolism. Biochem Pharmacol. 2002 Aug 15;64(4):553-64. Pubmed
- Waldmann A, Dorr B, Koschizke JW, Leitzmann C, Hahn A: Dietary intake of vitamin B6 and concentration of vitamin B6 in blood samples of German vegans. Public Health Nutr. 2006 Sep;9(6):779-84. Pubmed
- Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. Pubmed
8. Ornithine aminotransferase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P04181 
Gene: OAT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed
9. Ornithine decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P11926 
Gene: ODC1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Jackson LK, Goldsmith EJ, Phillips MA: X-ray structure determination of Trypanosoma brucei ornithine decarboxylase bound to D-ornithine and to G418: insights into substrate binding and ODC conformational flexibility. J Biol Chem. 2003 Jun 13;278(24):22037-43. Epub 2003 Apr 2. Pubmed
- Jackson LK, Brooks HB, Myers DP, Phillips MA: Ornithine decarboxylase promotes catalysis by binding the carboxylate in a buried pocket containing phenylalanine 397. Biochemistry. 2003 Mar 18;42(10):2933-40. Pubmed
- Jackson LK, Baldwin J, Akella R, Goldsmith EJ, Phillips MA: Multiple active site conformations revealed by distant site mutation in ornithine decarboxylase. Biochemistry. 2004 Oct 19;43(41):12990-9. Pubmed
- Khomutov AR: Inhibition of enzymes of polyamine biosynthesis by substrate-like O-substituted hydroxylamines. Biochemistry (Mosc). 2002 Oct;67(10):1159-67. Pubmed
- Myers DP, Jackson LK, Ipe VG, Murphy GE, Phillips MA: Long-range interactions in the dimer interface of ornithine decarboxylase are important for enzyme function. Biochemistry. 2001 Nov 6;40(44):13230-6. Pubmed
10. Kynurenine/alpha-aminoadipate aminotransferase mitochondrial
Pharmacological action: unknown
Actions: cofactor
Shows activity also towards tryptophan, aspartate and hydroxykinurenine
Organism class: human
UniProt ID: Q8N5Z0 
Gene: AADAT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Goh DL, Patel A, Thomas GH, Salomons GS, Schor DS, Jakobs C, Geraghty MT: Characterization of the human gene encoding alpha-aminoadipate aminotransferase (AADAT). Mol Genet Metab. 2002 Jul;76(3):172-80. Pubmed
- Cooper AJ: The role of glutamine transaminase K (GTK) in sulfur and alpha-keto acid metabolism in the brain, and in the possible bioactivation of neurotoxicants. Neurochem Int. 2004 Jun;44(8):557-77. Pubmed
11. 4-aminobutyrate aminotransferase, mitochondrial
Pharmacological action: unknown
Actions: inhibitor
Catalyzes the conversion of gamma-aminobutyrate and L- beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine
Organism class: human
UniProt ID: P80404 
Gene: ABAT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Storici P, De Biase D, Bossa F, Bruno S, Mozzarelli A, Peneff C, Silverman RB, Schirmer T: Structures of gamma-aminobutyric acid (GABA) aminotransferase, a pyridoxal 5’-phosphate, and [2Fe-2S] cluster-containing enzyme, complexed with gamma-ethynyl-GABA and with the antiepilepsy drug vigabatrin. J Biol Chem. 2004 Jan 2;279(1):363-73. Epub 2003 Oct 8. Pubmed
- Hwang IK, Yoo KY, Kim do H, Lee BH, Kwon YG, Won MH: Time course of changes in pyridoxal 5’-phosphate (vitamin B6 active form) and its neuroprotection in experimental ischemic damage. Exp Neurol. 2007 Jul;206(1):114-25. Epub 2007 Apr 24. Pubmed
- Sulaiman SA, Suliman FE, Barghouthi S: Kinetic studies on the inhibition of GABA-T by gamma-vinyl GABA and taurine. J Enzyme Inhib Med Chem. 2003 Aug;18(4):297-301. Pubmed
- Markova M, Peneff C, Hewlins MJ, Schirmer T, John RA: Determinants of substrate specificity in omega-aminotransferases. J Biol Chem. 2005 Oct 28;280(43):36409-16. Epub 2005 Aug 11. Pubmed
- Liu W, Peterson PE, Langston JA, Jin X, Zhou X, Fisher AJ, Toney MD: Kinetic and crystallographic analysis of active site mutants of Escherichia coli gamma-aminobutyrate aminotransferase. Biochemistry. 2005 Mar 1;44(8):2982-92. Pubmed
- Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. Pubmed
12. Pyridoxine-5'-phosphate oxidase
Pharmacological action: unknown
Actions: cofactor
Oxidizes PNP and PMP into pyridoxal 5'-phosphate (PLP)
Organism class: human
UniProt ID: Q9NVS9 
Gene: PNPO 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Gospe SM Jr: Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment. Curr Opin Neurol. 2006 Apr;19(2):148-53. Pubmed
- Hwang IK, Kim DW, Jung JY, Yoo KY, Cho JH, Kwon OS, Kang TC, Choi SY, Kim YS, Won MH: Age-dependent changes of pyridoxal phosphate synthesizing enzymes immunoreactivities and activities in the gerbil hippocampal CA1 region. Mech Ageing Dev. 2005 Dec;126(12):1322-30. Epub 2005 Oct 3. Pubmed
- Pearl PL, Hartka TR, Taylor J: Diagnosis and Treatment of Neurotransmitter Disorders. Curr Treat Options Neurol. 2006 Nov;8(6):441-450. Pubmed
- Hoffmann GF, Schmitt B, Windfuhr M, Wagner N, Strehl H, Bagci S, Franz AR, Mills PB, Clayton PT, Baumgartner MR, Steinmann B, Bast T, Wolf NI, Zschocke J: Pyridoxal 5’-phosphate may be curative in early-onset epileptic encephalopathy. J Inherit Metab Dis. 2007 Feb;30(1):96-9. Epub 2006 Dec 23. Pubmed
- Mills PB, Surtees RA, Champion MP, Beesley CE, Dalton N, Scambler PJ, Heales SJ, Briddon A, Scheimberg I, Hoffmann GF, Zschocke J, Clayton PT: Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5’-phosphate oxidase. Hum Mol Genet. 2005 Apr 15;14(8):1077-86. Epub 2005 Mar 16. Pubmed
13. Sphingosine-1-phosphate lyase 1
Pharmacological action: unknown
Actions: cofactor
Cleaves phosphorylated sphingoid bases (PSBs), such as sphingosine-1-phosphate, into fatty aldehydes and phosphoethanolamine. Elevates stress-induced ceramide production and apoptosis
Organism class: human
UniProt ID: O95470 
Gene: SGPL1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Ikeda M, Kihara A, Igarashi Y: Sphingosine-1-phosphate lyase SPL is an endoplasmic reticulum-resident, integral membrane protein with the pyridoxal 5’-phosphate binding domain exposed to the cytosol. Biochem Biophys Res Commun. 2004 Dec 3;325(1):338-43. Pubmed
- Bobbin RP: PPADS, an ATP antagonist, attenuates the effects of a moderately intense sound on cochlear mechanics. Hear Res. 2001 Jun;156(1-2):10-6. Pubmed
14. Tyrosine aminotransferase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P17735 
Gene: TAT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Biagini MR, Tozzi A, Marcucci R, Paniccia R, Fedi S, Milani S, Galli A, Ceni E, Capanni M, Manta R, Abbate R, Surrenti C: Hyperhomocysteinemia and hypercoagulability in primary biliary cirrhosis. World J Gastroenterol. 2006 Mar 14;12(10):1607-12. Pubmed
- Clayton TA, Lindon JC, Everett JR, Charuel C, Hanton G, Le Net JL, Provost JP, Nicholson JK: Hepatotoxin-induced hypertyrosinemia and its toxicological significance. Arch Toxicol. 2007 Mar;81(3):201-10. Epub 2006 Aug 11. Pubmed
- Kim SY, An JJ, Kim DW, Choi SH, Lee SH, Hwang SI, Kwon OS, Kang TC, Won MH, Cho SW, Park J, Eum WS, Lee KS, Choi SY: Tat-mediated protein transduction of human brain pyridoxine-5-P oxidase into PC12 cells. J Biochem Mol Biol. 2006 Jan 31;39(1):76-83. Pubmed
- Shaffer WA, Luong TN, Rothman SC, Kirsch JF: Quantitative chimeric analysis of six specificity determinants that differentiate Escherichia coli aspartate from tyrosine aminotransferase. Protein Sci. 2002 Dec;11(12):2848-59. Pubmed
- Venhorst J, ter Laak AM, Meijer M, van de Wetering I, Commandeur JN, Rooseboom M, Vermeulen NP: Modeling and molecular dynamics of glutamine transaminase K/cysteine conjugate beta-lyase. J Mol Graph Model. 2003 Sep;22(1):55-70. Pubmed
15. Kynurenine--oxoglutarate transaminase 1
Pharmacological action: unknown
Actions: cofactor
Catalyzes the irreversible transamination of the L- tryptophan metabolite L-kinurenine to form kynurenic acid (KA). Metabolizes the cysteine conjugates of certain halogenated alkenes and alkanes to form reactive metabolites. Catalyzes the beta- elimination of S-conjugates and Se-conjugates of L- (seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond
Organism class: human
UniProt ID: Q16773 
Gene: CCBL1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Cooper AJ, Bruschi SA, Anders MW: Toxic, halogenated cysteine S-conjugates and targeting of mitochondrial enzymes of energy metabolism. Biochem Pharmacol. 2002 Aug 15;64(4):553-64. Pubmed
- Mosca M, Croci C, Mostardini M, Breton J, Malyszko J, Avanzi N, Toma S, Benatti L, Gatti S: Tissue expression and translational control of rat kynurenine aminotransferase/glutamine transaminase K mRNAs. Biochim Biophys Acta. 2003 Jul 9;1628(1):1-10. Pubmed
- Zhang L, Cooper AJ, Krasnikov BF, Xu H, Bubber P, Pinto JT, Gibson GE, Hanigan MH: Cisplatin-induced toxicity is associated with platinum deposition in mouse kidney mitochondria in vivo and with selective inactivation of the alpha-ketoglutarate dehydrogenase complex in LLC-PK1 cells. Biochemistry. 2006 Jul 25;45(29):8959-71. Pubmed
- Venhorst J, ter Laak AM, Meijer M, van de Wetering I, Commandeur JN, Rooseboom M, Vermeulen NP: Modeling and molecular dynamics of glutamine transaminase K/cysteine conjugate beta-lyase. J Mol Graph Model. 2003 Sep;22(1):55-70. Pubmed
- Cooper AJ, Pinto JT: Cysteine S-conjugate beta-lyases. Amino Acids. 2006 Feb;30(1):1-15. Epub 2006 Feb 6. Pubmed
16. Threonine synthase-like 1
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q8IYQ7 
Gene: THNSL1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
17. Glycogen phosphorylase, liver form
Pharmacological action: unknown
Actions: cofactor
Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties
Organism class: human
UniProt ID: P06737 
Gene: PYGL 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Beauchamp NJ, Taybert J, Champion MP, Layet V, Heinz-Erian P, Dalton A, Tanner MS, Pronicka E, Sharrard MJ: High frequency of missense mutations in glycogen storage disease type VI. J Inherit Metab Dis. 2007 Oct;30(5):722-34. Epub 2007 Aug 21. Pubmed
18. Serine palmitoyltransferase 2
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: O15270 
Gene: SPTLC2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Hanada K: Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. Biochim Biophys Acta. 2003 Jun 10;1632(1-3):16-30. Pubmed
- Tamura K, Mitsuhashi N, Hara-Nishimura I, Imai H: Characterization of an Arabidopsis cDNA encoding a subunit of serine palmitoyltransferase, the initial enzyme in sphingolipid biosynthesis. Plant Cell Physiol. 2001 Nov;42(11):1274-81. Pubmed
- Gable K, Han G, Monaghan E, Bacikova D, Natarajan M, Williams R, Dunn TM: Mutations in the yeast LCB1 and LCB2 genes, including those corresponding to the hereditary sensory neuropathy type I mutations, dominantly inactivate serine palmitoyltransferase. J Biol Chem. 2002 Mar 22;277(12):10194-200. Epub 2002 Jan 7. Pubmed
- Ikushiro H, Hayashi H, Kagamiyama H: A water-soluble homodimeric serine palmitoyltransferase from Sphingomonas paucimobilis EY2395T strain. Purification, characterization, cloning, and overproduction. J Biol Chem. 2001 May 25;276(21):18249-56. Epub 2001 Mar 12. Pubmed
- Tamura K, Nishiura H, Mori J, Imai H: Cloning and characterization of a cDNA encoding serine palmitoyltransferase in Arabidopsis thaliana. Biochem Soc Trans. 2000 Dec;28(6):745-7. Pubmed
19. Cysteine sulfinic acid decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9Y600 
Gene: CSAD 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Skoldberg F, Rorsman F, Perheentupa J, Landin-Olsson M, Husebye ES, Gustafsson J, Kampe O: Analysis of antibody reactivity against cysteine sulfinic acid decarboxylase, a pyridoxal phosphate-dependent enzyme, in endocrine autoimmune disease. J Clin Endocrinol Metab. 2004 Apr;89(4):1636-40. Pubmed
20. Histidine decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P19113 
Gene: HDC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Skoldberg F, Portela-Gomes GM, Grimelius L, Nilsson G, Perheentupa J, Betterle C, Husebye ES, Gustafsson J, Ronnblom A, Rorsman F, Kampe O: Histidine decarboxylase, a pyridoxal phosphate-dependent enzyme, is an autoantigen of gastric enterochromaffin-like cells. J Clin Endocrinol Metab. 2003 Apr;88(4):1445-52. Pubmed
- Moya-Garcia AA, Pino-Angeles A, Sanchez-Jimenez F: New structural insights to help in the search for selective inhibitors of mammalian pyridoxal 5’-phosphate-dependent histidine decarboxylase . 4. Synthesis, metabolism and release of histamine. Inflamm Res. 2006 Apr;55 Suppl 1:S55-6. Pubmed
- Landete JM, Pardo I, Ferrer S: Histamine, histidine, and growth-phase mediated regulation of the histidine decarboxylase gene in lactic acid bacteria isolated from wine. FEMS Microbiol Lett. 2006 Jul;260(1):84-90. Pubmed
- Skoldberg F, Rorsman F, Perheentupa J, Landin-Olsson M, Husebye ES, Gustafsson J, Kampe O: Analysis of antibody reactivity against cysteine sulfinic acid decarboxylase, a pyridoxal phosphate-dependent enzyme, in endocrine autoimmune disease. J Clin Endocrinol Metab. 2004 Apr;89(4):1636-40. Pubmed
- Graham DE, Xu H, White RH: Methanococcus jannaschii uses a pyruvoyl-dependent arginine decarboxylase in polyamine biosynthesis. J Biol Chem. 2002 Jun 28;277(26):23500-7. Epub 2002 Apr 29. Pubmed
21. Arginine decarboxylase
Pharmacological action: unknown
Actions: cofactor
Decarboxylates L-arginine to agmatine. Truncated splice isoforms probably lack activity
Organism class: human
UniProt ID: Q96A70 
Gene: ADC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Graham DE, Xu H, White RH: Methanococcus jannaschii uses a pyruvoyl-dependent arginine decarboxylase in polyamine biosynthesis. J Biol Chem. 2002 Jun 28;277(26):23500-7. Epub 2002 Apr 29. Pubmed
- Patel CN, Adcock RS, Sell KG, Oliveira MA: Crystallization, X-ray diffraction and oligomeric characterization of arginine decarboxylase from Yersinia pestis, a key polyamine biosynthetic enzyme. Acta Crystallogr D Biol Crystallogr. 2004 Dec;60(Pt 12 Pt 2):2396-8. Epub 2004 Nov 26. Pubmed
- Kidron H, Repo S, Johnson MS, Salminen TA: Functional classification of amino acid decarboxylases from the alanine racemase structural family by phylogenetic studies. Mol Biol Evol. 2007 Jan;24(1):79-89. Epub 2006 Sep 22. Pubmed
- Arena ME, Manca de Nadra MC: Biogenic amine production by Lactobacillus. J Appl Microbiol. 2001 Feb;90(2):158-62. Pubmed
- Shah R, Akella R, Goldsmith EJ, Phillips MA: X-ray structure of Paramecium bursaria Chlorella virus arginine decarboxylase: insight into the structural basis for substrate specificity. Biochemistry. 2007 Mar 13;46(10):2831-41. Epub 2007 Feb 17. Pubmed
22. L-serine dehydratase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P20132 
Gene: SDS 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Sun L, Bartlam M, Liu Y, Pang H, Rao Z: Crystal structure of the pyridoxal-5’-phosphate-dependent serine dehydratase from human liver. Protein Sci. 2005 Mar;14(3):791-8. Epub 2005 Feb 2. Pubmed
- Yamada T, Komoto J, Takata Y, Ogawa H, Pitot HC, Takusagawa F: Crystal structure of serine dehydratase from rat liver. Biochemistry. 2003 Nov 11;42(44):12854-65. Pubmed
- Sun L, Li X, Dong Y, Yang M, Liu Y, Han X, Zhang X, Pang H, Rao Z: Crystallization and preliminary crystallographic analysis of human serine dehydratase. Acta Crystallogr D Biol Crystallogr. 2003 Dec;59(Pt 12):2297-9. Epub 2003 Nov 27. Pubmed
- Ogawa H, Gomi T, Nishizawa M, Hayakawa Y, Endo S, Hayashi K, Ochiai H, Takusagawa F, Pitot HC, Mori H, Sakurai H, Koizumi K, Saiki I, Oda H, Fujishita T, Miwa T, Maruyama M, Kobayashi M: Enzymatic and biochemical properties of a novel human serine dehydratase isoform. Biochim Biophys Acta. 2006 May;1764(5):961-71. Epub 2006 Mar 20. Pubmed
- Cicchillo RM, Baker MA, Schnitzer EJ, Newman EB, Krebs C, Booker SJ: Escherichia coli L-serine deaminase requires a [4Fe-4S] cluster in catalysis. J Biol Chem. 2004 Jul 30;279(31):32418-25. Epub 2004 May 19. Pubmed
23. 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: O75600 
Gene: GCAT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Schmidt A, Sivaraman J, Li Y, Larocque R, Barbosa JA, Smith C, Matte A, Schrag JD, Cygler M: Three-dimensional structure of 2-amino-3-ketobutyrate CoA ligase from Escherichia coli complexed with a PLP-substrate intermediate: inferred reaction mechanism. Biochemistry. 2001 May 1;40(17):5151-60. Pubmed
24. Glycine dehydrogenase [decarboxylating], mitochondrial
Pharmacological action: unknown
Actions: cofactor
The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein
Organism class: human
UniProt ID: P23378 
Gene: GLDC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Nakai T, Nakagawa N, Maoka N, Masui R, Kuramitsu S, Kamiya N: Structure of P-protein of the glycine cleavage system: implications for nonketotic hyperglycinemia. EMBO J. 2005 Apr 20;24(8):1523-36. Epub 2005 Mar 24. Pubmed
- Igamberdiev AU, Ivlev AA, Bykova NV, Threlkeld CN, Lea PJ, Gardestrom P: Decarboxylation of glycine contributes to carbon isotope fractionation in photosynthetic organisms. Photosynth Res. 2001;67(3):177-84. Pubmed
25. Alanine aminotransferase 1
Pharmacological action: unknown
Actions: cofactor
Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles
Organism class: human
UniProt ID: P24298 
Gene: GPT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Cheung PY, Fong CC, Ng KT, Lam WC, Leung YC, Tsang CW, Yang M, Wong MS: Interaction between pyridoxal kinase and pyridoxal-5-phosphate-dependent enzymes. J Biochem (Tokyo). 2003 Nov;134(5):731-8. Pubmed
- Halfon P, Imbert-Bismut F, Messous D, Antoniotti G, Benchetrit D, Cart-Lamy P, Delaporte G, Doutheau D, Klump T, Sala M, Thibaud D, Trepo E, Thabut D, Myers RP, Poynard T: A prospective assessment of the inter-laboratory variability of biochemical markers of fibrosis (FibroTest) and activity (ActiTest) in patients with chronic liver disease. Comp Hepatol. 2002 Dec 30;1(1):3. Pubmed
- Inubushi T, Takasawa T, Tuboi Y, Watanabe N, Aki K, Katunuma N: Changes of glucose metabolism and skin-collagen neogenesis in vitamin B6 deficiency. Biofactors. 2005;23(2):59-67. Pubmed
- Baines CJ, McKeown-Eyssen GE, Riley N, Cole DE, Marshall L, Loescher B, Jazmaji V: Case-control study of multiple chemical sensitivity, comparing haematology, biochemistry, vitamins and serum volatile organic compound measures. Occup Med (Lond). 2004 Sep;54(6):408-18. Epub 2004 Sep 3. Pubmed
- Beranek M, Drsata J, Palicka V: Inhibitory effect of glycation on catalytic activity of alanine aminotransferase. Mol Cell Biochem. 2001 Feb;218(1-2):35-9. Pubmed
26. Phosphoserine aminotransferase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9Y617 
Gene: PSAT1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Battchikova N, Himanen JP, Ahjolahti M, Korpela T: Phosphoserine aminotransferase from Bacillus circulans subsp. alkalophilus: purification, gene cloning and sequencing. Biochim Biophys Acta. 1996 Jul 18;1295(2):187-94. Pubmed
- Hester G, Stark W, Moser M, Kallen J, Markovic-Housley Z, Jansonius JN: Crystal structure of phosphoserine aminotransferase from Escherichia coli at 2.3 A resolution: comparison of the unligated enzyme and a complex with alpha-methyl-l-glutamate. J Mol Biol. 1999 Feb 26;286(3):829-50. Pubmed
- Kapetaniou EG, Thanassoulas A, Dubnovitsky AP, Nounesis G, Papageorgiou AC: Effect of pH on the structure and stability of Bacillus circulans ssp. alkalophilus phosphoserine aminotransferase: thermodynamic and crystallographic studies. Proteins. 2006 Jun 1;63(4):742-53. Pubmed
- Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed
27. 5-aminolevulinate synthase, nonspecific, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P13196 
Gene: ALAS1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Nakamura I, Isobe N, Nakamura N, Kamihara T, Fukui S: Mechanism of thiamine-induced respiratory deficiency in Saccharomyces carlsbergensis. J Bacteriol. 1981 Sep;147(3):954-61. Pubmed
- Harigae H, Furuyama K, Kudo K, Hayashi N, Yamamoto M, Sassa S, Sasaki T: A novel mutation of the erythroid-specific gamma-Aminolevulinate synthase gene in a patient with non-inherited pyridoxine-responsive sideroblastic anemia. Am J Hematol. 1999 Oct;62(2):112-4. Pubmed
- Shoolingin-Jordan PM, Al-Daihan S, Alexeev D, Baxter RL, Bottomley SS, Kahari ID, Roy I, Sarwar M, Sawyer L, Wang SF: 5-Aminolevulinic acid synthase: mechanism, mutations and medicine. Biochim Biophys Acta. 2003 Apr 11;1647(1-2):361-6. Pubmed
28. Serine--pyruvate aminotransferase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P21549 
Gene: AGXT 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Coulter-Mackie MB, Lian Q: Consequences of missense mutations for dimerization and turnover of alanine:glyoxylate aminotransferase: study of a spectrum of mutations. Mol Genet Metab. 2006 Dec;89(4):349-59. Epub 2006 Sep 12. Pubmed
29. Pyridoxal phosphate phosphatase
Pharmacological action: unknown
Actions: cofactor
Pyridoxal phosphate phosphatase. Has some activity towards pyridoxal 5'-phosphate (PLP), pyridoxine 5'-phosphate (PMP) and Pyridoxine 5'-phosphate (PNP), with a highest activity with PLP followed by PNP
Organism class: human
UniProt ID: Q96GD0 
Gene: PDXP 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Jang YM, Kim DW, Kang TC, Won MH, Baek NI, Moon BJ, Choi SY, Kwon OS: Human pyridoxal phosphatase. Molecular cloning, functional expression, and tissue distribution. J Biol Chem. 2003 Dec 12;278(50):50040-6. Epub 2003 Sep 30. Pubmed
- McCarty MF: Increased homocyst(e)ine associated with smoking, chronic inflammation, and aging may reflect acute-phase induction of pyridoxal phosphatase activity. Med Hypotheses. 2000 Oct;55(4):289-93. Pubmed
- Hwang IK, Yoo KY, Kim do H, Lee BH, Kwon YG, Won MH: Time course of changes in pyridoxal 5’-phosphate (vitamin B6 active form) and its neuroprotection in experimental ischemic damage. Exp Neurol. 2007 Jul;206(1):114-25. Epub 2007 Apr 24. Pubmed
- Kim DW, Eum WS, Choi HS, Kim SY, An JJ, Lee SH, Sohn EJ, Hwang SI, Kwon OS, Kang TC, Won MH, Cho SW, Lee KS, Park J, Choi SY: Human brain pyridoxal-5’-phosphate phosphatase: production and characterization of monoclonal antibodies. J Biochem Mol Biol. 2005 Nov 30;38(6):703-8. Pubmed
- Tirrell IM, Wall JL, Daley CJ, Denial SJ, Tennis FG, Galens KG, O’Handley SF: YZGD from Paenibacillus thiaminolyticus, a pyridoxal phosphatase of the HAD (haloacid dehalogenase) superfamily and a versatile member of the Nudix (nucleoside diphosphate x) hydrolase superfamily. Biochem J. 2006 Mar 15;394(Pt 3):665-74. Pubmed
30. Serine palmitoyltransferase 1
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: O15269 
Gene: SPTLC1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Hanada K: Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. Biochim Biophys Acta. 2003 Jun 10;1632(1-3):16-30. Pubmed
- Gable K, Han G, Monaghan E, Bacikova D, Natarajan M, Williams R, Dunn TM: Mutations in the yeast LCB1 and LCB2 genes, including those corresponding to the hereditary sensory neuropathy type I mutations, dominantly inactivate serine palmitoyltransferase. J Biol Chem. 2002 Mar 22;277(12):10194-200. Epub 2002 Jan 7. Pubmed
- Ikushiro H, Hayashi H, Kagamiyama H: A water-soluble homodimeric serine palmitoyltransferase from Sphingomonas paucimobilis EY2395T strain. Purification, characterization, cloning, and overproduction. J Biol Chem. 2001 May 25;276(21):18249-56. Epub 2001 Mar 12. Pubmed
31. Cystathionine gamma-lyase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P32929 
Gene: CTH 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Yamagata S, Akamatsu T, Iwama T: Immobilization of Saccharomyces cerevisiae cystathionine gamma-lyase and application of the product to cystathionine synthesis. Appl Environ Microbiol. 2004 Jun;70(6):3766-8. Pubmed
- Bertoldi M, Cellini B, Laurents DV, Borri Voltattorni C: Folding pathway of the pyridoxal 5’-phosphate C-S lyase MalY from Escherichia coli. Biochem J. 2005 Aug 1;389(Pt 3):885-98. Pubmed
- Lowicka E, Beltowski J: Hydrogen sulfide (H2S) – the third gas of interest for pharmacologists. Pharmacol Rep. 2007 Jan-Feb;59(1):4-24. Pubmed
- Lima CP, Davis SR, Mackey AD, Scheer JB, Williamson J, Gregory JF 3rd: Vitamin B-6 deficiency suppresses the hepatic transsulfuration pathway but increases glutathione concentration in rats fed AIN-76A or AIN-93G diets. J Nutr. 2006 Aug;136(8):2141-7. Pubmed
- Okuno T, Kubota T, Kuroda T, Ueno H, Nakamuro K: Contribution of enzymic alpha, gamma-elimination reaction in detoxification pathway of selenomethionine in mouse liver. Toxicol Appl Pharmacol. 2001 Oct 1;176(1):18-23. Pubmed
32. Branched-chain-amino-acid aminotransferase, cytosolic
Pharmacological action: unknown
Actions: cofactor
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine
Organism class: human
UniProt ID: P54687 
Gene: BCAT1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
33. Branched-chain-amino-acid aminotransferase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids
Organism class: human
UniProt ID: O15382 
Gene: BCAT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
34. Proline synthetase co-transcribed bacterial homolog protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: O94903 
Gene: PROSC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
35. Formimidoyltransferase-cyclodeaminase
Pharmacological action: unknown
Actions: cofactor
Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool
Organism class: human
UniProt ID: O95954 
Gene: FTCD 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
36. Aspartate aminotransferase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: P00505 
Gene: GOT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Mavrides C, Orr W: Multispecific aspartate and aromatic amino acid aminotransferases in Escherichia coli. J Biol Chem. 1975 Jun 10;250(11):4128-33. Pubmed
37. Glycogen phosphorylase, brain form
Pharmacological action: unknown
Actions: cofactor
Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties
Organism class: human
UniProt ID: P11216 
Gene: PYGB 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
38. Glycogen phosphorylase, muscle form
Pharmacological action: unknown
Actions: cofactor
Phosphorylase is an important allosteric enzyme in carbohydrate metabolism. Enzymes from different sources differ in their regulatory mechanisms and in their natural substrates. However, all known phosphorylases share catalytic and structural properties
Organism class: human
UniProt ID: P11217 
Gene: PYGM 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Livanova NB, Chebotareva NA, Eronina TB, Kurganov BI: Pyridoxal 5’-phosphate as a catalytic and conformational cofactor of muscle glycogen phosphorylase B. Biochemistry (Mosc). 2002 Oct;67(10):1089-98. Pubmed
- Kurganov BI, Kornilaev BA, Chebotareva NA, Malikov VP, Orlov VN, Lyubarev AE, Livanova NB: Dissociative mechanism of thermal denaturation of rabbit skeletal muscle glycogen phosphorylase b. Biochemistry. 2000 Oct 31;39(43):13144-52. Pubmed
- Okada M, Shibuya M, Yamamoto E, Murakami Y: Effect of diabetes on vitamin B6 requirement in experimental animals. Diabetes Obes Metab. 1999 Jul;1(4):221-5. Pubmed
- Okada M, Goda H, Kondo Y, Murakami Y, Shibuya M: Effect of exercise on the metabolism of vitamin B6 and some PLP-dependent enzymes in young rats fed a restricted vitamin B6 diet. J Nutr Sci Vitaminol (Tokyo). 2001 Apr;47(2):116-21. Pubmed
- Geremia S, Campagnolo M, Schinzel R, Johnson LN: Enzymatic catalysis in crystals of Escherichia coli maltodextrin phosphorylase. J Mol Biol. 2002 Sep 13;322(2):413-23. Pubmed
39. Aromatic-L-amino-acid decarboxylase
Pharmacological action: unknown
Actions: cofactor
Catalyzes the decarboxylation of L-3,4- dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine
Organism class: human
UniProt ID: P20711 
Gene: DDC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Rahman MK, Nagatsu T, Sakurai T, Hori S, Abe M, Matsuda M: Effect of pyridoxal phosphate deficiency on aromatic L-amino acid decarboxylase activity with L-DOPA and L-5-hydroxytryptophan as substrates in rats. Jpn J Pharmacol. 1982 Oct;32(5):803-11. Pubmed
- Rorsman F, Husebye ES, Winqvist O, Bjork E, Karlsson FA, Kampe O: Aromatic-L-amino-acid decarboxylase, a pyridoxal phosphate-dependent enzyme, is a beta-cell autoantigen. Proc Natl Acad Sci U S A. 1995 Sep 12;92(19):8626-9. Pubmed
- Bertoldi M, Borri Voltattorni C: Reaction of dopa decarboxylase with L-aromatic amino acids under aerobic and anaerobic conditions. Biochem J. 2000 Dec 1;352 Pt 2:533-8. Pubmed
- Allen GF, Neergheen V, Oppenheim M, Fitzgerald JC, Footitt E, Hyland K, Clayton PT, Land JM, Heales SJ: Pyridoxal 5’-phosphate deficiency causes a loss of aromatic L-amino acid decarboxylase in patients and human neuroblastoma cells, implications for aromatic L-amino acid decarboxylase and vitamin B(6) deficiency states. J Neurochem. 2010 Jul;114(1):87-96. Epub 2010 Apr 9. Pubmed
40. Serine hydroxymethyltransferase, mitochondrial
Pharmacological action: unknown
Actions: cofactor
Interconversion of serine and glycine
Organism class: human
UniProt ID: P34897 
Gene: SHMT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Trakatellis A, Dimitriadou A, Exindari M, Christodoulou D, Malissiovas N, Antoniadis A, Haitoglou K: Effect of combination of deoxypyridoxine with known anti-proliferative or immunosuppressive agents on lymphocyte serine hydroxymethyltransferase. Postgrad Med J. 1994;70 Suppl 1:S89-92. Pubmed
- Jagath JR, Sharma B, Rao NA, Savithri HS: The role of His-134, -147, and -150 residues in subunit assembly, cofactor binding, and catalysis of sheep liver cytosolic serine hydroxymethyltransferase. J Biol Chem. 1997 Sep 26;272(39):24355-62. Pubmed
- Bourguignon J, Neuburger M, Douce R: Resolution and characterization of the glycine-cleavage reaction in pea leaf mitochondria. Properties of the forward reaction catalysed by glycine decarboxylase and serine hydroxymethyltransferase. Biochem J. 1988 Oct 1;255(1):169-78. Pubmed
41. Growth-inhibiting protein 18
Pharmacological action: unknown
Actions: cofactor
L-aspartate + 2-oxoglutarate = oxaloacetate + L-glutamate
Organism class: human
UniProt ID: Q2TU84 
Gene: GIG18 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
42. GAD1 protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q49AK1 
Gene: GAD1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Bennett CL, Huynh HM, Chance PF, Glass IA, Gospe SM Jr: Genetic heterogeneity for autosomal recessive pyridoxine-dependent seizures. Neurogenetics. 2005 Sep;6(3):143-9. Epub 2005 Aug 2. Pubmed
- Vassort C, Riviere M, Bruneau G, Gros F, Thibault J, Levan G, Szpirer J, Szpirer C: Assignment of the rat genes coding for dopa decarboxylase (DDC) and glutamic acid decarboxylases (GAD1 and GAD2). Mamm Genome. 1993;4(4):202-6. Pubmed
- Cormier-Daire V, Dagoneau N, Nabbout R, Burglen L, Penet C, Soufflet C, Desguerre I, Munnich A, Dulac O: A gene for pyridoxine-dependent epilepsy maps to chromosome 5q31. Am J Hum Genet. 2000 Oct;67(4):991-3. Epub 2000 Sep 7. Pubmed
43. Serine hydroxymethyltransferase 2
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q53ET4 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Mehta R, Shangari N, O’brien PJ: Preventing cell death induced by carbonyl stress, oxidative stress or mitochondrial toxins with vitamin B anti-AGE agents. Mol Nutr Food Res. 2007 Oct 4;. Pubmed
44. Serine hydroxymethyltransferase 1
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q53ET7 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Bayes M, Rabasseda X, Prous JR: Gateways to clinical trials. Methods Find Exp Clin Pharmacol. 2007 Jun;29(5):359-73. Pubmed
- Bleie O, Semb AG, Grundt H, Nordrehaug JE, Vollset SE, Ueland PM, Nilsen DW, Bakken AM, Refsum H, Nygard OK: Homocysteine-lowering therapy does not affect inflammatory markers of atherosclerosis in patients with stable coronary artery disease. J Intern Med. 2007 Aug;262(2):244-53. Pubmed
45. Selenocysteine lyase variant
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q59FK2 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Heidenreich T, Wollers S, Mendel RR, Bittner F: Characterization of the NifS-like domain of ABA3 from Arabidopsis thaliana provides insight into the mechanism of molybdenum cofactor sulfuration. J Biol Chem. 2005 Feb 11;280(6):4213-8. Epub 2004 Nov 22. Pubmed
46. Brain glycogen phosphorylase variant
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q59GM9 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
47. Ornithine aminotransferase variant
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q59HE2 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
48. SHMT2 protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q5BJF5 
Gene: SHMT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
49. Hypothetical protein DKFZp686P09201
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q5HYG8 
Gene: DKFZp686P09201 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
50. Migration-inducing protein 4
Pharmacological action: unknown
Actions: cofactor
Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO(2)
Organism class: human
UniProt ID: Q5JAM2 
Gene: ALAS1
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Ferreira GC, Zhang JS: Mechanism of 5-aminolevulinate synthase and the role of the protein environment in controlling the cofactor chemistry. Cell Mol Biol (Noisy-le-grand). 2002 Dec;48(8):827-33. Pubmed
- Choi HP, Hong JW, Rhee KH, Sung HC: Cloning, expression, and characterization of 5-aminolevulinic acid synthase from Rhodopseudomonas palustris KUGB306. FEMS Microbiol Lett. 2004 Jul 15;236(2):175-81. Pubmed
- Zhang J, Ferreira GC: Transient state kinetic investigation of 5-aminolevulinate synthase reaction mechanism. J Biol Chem. 2002 Nov 22;277(47):44660-9. Epub 2002 Aug 20. Pubmed
- Turbeville TD, Zhang J, Hunter GA, Ferreira GC: Histidine 282 in 5-aminolevulinate synthase affects substrate binding and catalysis. Biochemistry. 2007 May 22;46(20):5972-81. Epub 2007 May 1. Pubmed
- Zhang J, Cheltsov AV, Ferreira GC: Conversion of 5-aminolevulinate synthase into a more active enzyme by linking the two subunits: spectroscopic and kinetic properties. Protein Sci. 2005 May;14(5):1190-200. Pubmed
51. Phosphorylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q5JWL9 
Gene: PYGB 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
52. Glutamate decarboxylase 2
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q5VZ30 
Gene: GAD2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Bennett CL, Huynh HM, Chance PF, Glass IA, Gospe SM Jr: Genetic heterogeneity for autosomal recessive pyridoxine-dependent seizures. Neurogenetics. 2005 Sep;6(3):143-9. Epub 2005 Aug 2. Pubmed
- Vassort C, Riviere M, Bruneau G, Gros F, Thibault J, Levan G, Szpirer J, Szpirer C: Assignment of the rat genes coding for dopa decarboxylase (DDC) and glutamic acid decarboxylases (GAD1 and GAD2). Mamm Genome. 1993;4(4):202-6. Pubmed
- Cormier-Daire V, Dagoneau N, Nabbout R, Burglen L, Penet C, Soufflet C, Desguerre I, Munnich A, Dulac O: A gene for pyridoxine-dependent epilepsy maps to chromosome 5q31. Am J Hum Genet. 2000 Oct;67(4):991-3. Epub 2000 Sep 7. Pubmed
53. Putative L-Dopa decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q5W5T9 
Gene: DDC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
54. DDC protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q6IBS8 
Gene: DDC 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Tan EK, Cheah SY, Fook-Chong S, Yew K, Chandran VR, Lum SY, Yi Z: Functional COMT variant predicts response to high dose pyridoxine in Parkinson’s disease. Am J Med Genet B Neuropsychiatr Genet. 2005 Aug 5;137(1):1-4. Pubmed
- Bertoldi M, Borri Voltattorni C: Reaction of dopa decarboxylase with L-aromatic amino acids under aerobic and anaerobic conditions. Biochem J. 2000 Dec 1;352 Pt 2:533-8. Pubmed
- Yee RE, Cheng DW, Huang SC, Namavari M, Satyamurthy N, Barrio JR: Blood-brain barrier and neuronal membrane transport of 6-[18F]fluoro-L-DOPA. Biochem Pharmacol. 2001 Nov 15;62(10):1409-15. Pubmed
- Skoldberg F, Portela-Gomes GM, Grimelius L, Nilsson G, Perheentupa J, Betterle C, Husebye ES, Gustafsson J, Ronnblom A, Rorsman F, Kampe O: Histidine decarboxylase, a pyridoxal phosphate-dependent enzyme, is an autoantigen of gastric enterochromaffin-like cells. J Clin Endocrinol Metab. 2003 Apr;88(4):1445-52. Pubmed
- Skoldberg F, Rorsman F, Perheentupa J, Landin-Olsson M, Husebye ES, Gustafsson J, Kampe O: Analysis of antibody reactivity against cysteine sulfinic acid decarboxylase, a pyridoxal phosphate-dependent enzyme, in endocrine autoimmune disease. J Clin Endocrinol Metab. 2004 Apr;89(4):1636-40. Pubmed
55. KIAA0251 protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q6P996 
Gene: PDXDC1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
56. Kynurenine--oxoglutarate transaminase 3
Pharmacological action: unknown
Actions: cofactor
Catalyzes the irreversible transamination of the L- tryptophan metabolite L-kinurenine to form kynurenic acid (KA). May catalyze the beta-elimination of S-conjugates and Se- conjugates of L-(seleno)cysteine, resulting in the cleavage of the C-S or C-Se bond
Organism class: human
UniProt ID: Q6YP21 
Gene: CCBL2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
57. CDNA FLJ46802 fis, clone TRACH3032150, moderately similar to Cysteine sulfinic acid decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q6ZQY3 
Gene: GADL1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
58. Selenocysteine lyase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q96I15 
Gene: SCLY
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed
59. Immunoglobulin superfamily member 10
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q6WRI0 
Gene: IGSF10
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Berman HM, Westbrook J, Feng Z, Gilliland G, Bhat TN, Weissig H, Shindyalov IN, Bourne PE: The Protein Data Bank. Nucleic Acids Res. 2000 Jan 1;28(1):235-42. Pubmed
60. Alanine--glyoxylate aminotransferase 2-like 2
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q8IUZ5 
Gene: AGXT2L2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
61. Glutamate decarboxylase 1
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q8IVA8 
Gene: GAD1 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Bennett CL, Huynh HM, Chance PF, Glass IA, Gospe SM Jr: Genetic heterogeneity for autosomal recessive pyridoxine-dependent seizures. Neurogenetics. 2005 Sep;6(3):143-9. Epub 2005 Aug 2. Pubmed
- Vassort C, Riviere M, Bruneau G, Gros F, Thibault J, Levan G, Szpirer J, Szpirer C: Assignment of the rat genes coding for dopa decarboxylase (DDC) and glutamic acid decarboxylases (GAD1 and GAD2). Mamm Genome. 1993;4(4):202-6. Pubmed
- Cormier-Daire V, Dagoneau N, Nabbout R, Burglen L, Penet C, Soufflet C, Desguerre I, Munnich A, Dulac O: A gene for pyridoxine-dependent epilepsy maps to chromosome 5q31. Am J Hum Genet. 2000 Oct;67(4):991-3. Epub 2000 Sep 7. Pubmed
62. SHMT2 protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q8N1A5 
Gene: SHMT2
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Bhavani S, Trivedi V, Jala VR, Subramanya HS, Kaul P, Prakash V, Appaji Rao N, Savithri HS: Role of Lys-226 in the catalytic mechanism of Bacillus stearothermophilus serine hydroxymethyltransferase—crystal structure and kinetic studies. Biochemistry. 2005 May 10;44(18):6929-37. Pubmed
- Trivedi V, Gupta A, Jala VR, Saravanan P, Rao GS, Rao NA, Savithri HS, Subramanya HS: Crystal structure of binary and ternary complexes of serine hydroxymethyltransferase from Bacillus stearothermophilus: insights into the catalytic mechanism. J Biol Chem. 2002 May 10;277(19):17161-9. Epub 2002 Feb 27. Pubmed
- Perry C, Yu S, Chen J, Matharu KS, Stover PJ: Effect of vitamin B6 availability on serine hydroxymethyltransferase in MCF-7 cells. Arch Biochem Biophys. 2007 Jun 1;462(1):21-7. Epub 2007 Apr 20. Pubmed
- Rajaram V, Bhavani BS, Kaul P, Prakash V, Appaji Rao N, Savithri HS, Murthy MR: Structure determination and biochemical studies on Bacillus stearothermophilus E53Q serine hydroxymethyltransferase and its complexes provide insights on function and enzyme memory. FEBS J. 2007 Aug;274(16):4148-60. Epub 2007 Jul 25. Pubmed
- Mukherjee M, Sievers SA, Brown MT, Johnson PJ: Identification and biochemical characterization of serine hydroxymethyl transferase in the hydrogenosome of Trichomonas vaginalis. Eukaryot Cell. 2006 Dec;5(12):2072-8. Epub 2006 Sep 15. Pubmed
63. Aminolevulinate, delta-, synthase 2
Pharmacological action: unknown
Actions: cofactor
Succinyl-CoA + glycine = 5-aminolevulinate + CoA + CO(2)
Organism class: human
UniProt ID: Q8N6H3 
Gene: ALAS2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Shoolingin-Jordan PM, Al-Daihan S, Alexeev D, Baxter RL, Bottomley SS, Kahari ID, Roy I, Sarwar M, Sawyer L, Wang SF: 5-Aminolevulinic acid synthase: mechanism, mutations and medicine. Biochim Biophys Acta. 2003 Apr 11;1647(1-2):361-6. Pubmed
- Choi HP, Hong JW, Rhee KH, Sung HC: Cloning, expression, and characterization of 5-aminolevulinic acid synthase from Rhodopseudomonas palustris KUGB306. FEMS Microbiol Lett. 2004 Jul 15;236(2):175-81. Pubmed
- Heller T, Hochstetter V, Basler M, Borck V: [Vitamin B6-sensitive hereditary sideroblastic anemia] Dtsch Med Wochenschr. 2004 Jan 23;129(4):141-4. Pubmed
- Clayton PT: B6-responsive disorders: a model of vitamin dependency. J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):317-26. Pubmed
- Astner I, Schulze JO, van den Heuvel J, Jahn D, Schubert WD, Heinz DW: Crystal structure of 5-aminolevulinate synthase, the first enzyme of heme biosynthesis, and its link to XLSA in humans. EMBO J. 2005 Sep 21;24(18):3166-77. Epub 2005 Aug 25. Pubmed
64. Alanine aminotransferase 2
Pharmacological action: unknown
Actions: cofactor
L-alanine + 2-oxoglutarate = pyruvate + L- glutamate
Organism class: human
UniProt ID: Q8TD30 
Gene: GPT2 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Welch S: Comparative studies on the human glutamate-pyruvate transaminase phenotypes—GPT 1, GPT 2-1, GPT 2. Humangenetik. 1975 Sep 20;30(3):237-49. Pubmed
65. Molybdenum cofactor sulfurase
Pharmacological action: unknown
Actions: cofactor
Sulfurates the molybdenum cofactor. Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form
Organism class: human
UniProt ID: Q96EN8 
Gene: MOCOS 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Heidenreich T, Wollers S, Mendel RR, Bittner F: Characterization of the NifS-like domain of ABA3 from Arabidopsis thaliana provides insight into the mechanism of molybdenum cofactor sulfuration. J Biol Chem. 2005 Feb 11;280(6):4213-8. Epub 2004 Nov 22. Pubmed
- Mendel RR, Bittner F: Cell biology of molybdenum. Biochim Biophys Acta. 2006 Jul;1763(7):621-35. Epub 2006 May 12. Pubmed
- Anantharaman V, Aravind L: MOSC domains: ancient, predicted sulfur-carrier domains, present in diverse metal-sulfur cluster biosynthesis proteins including Molybdenum cofactor sulfurases. FEMS Microbiol Lett. 2002 Jan 22;207(1):55-61. Pubmed
- Bittner F, Oreb M, Mendel RR: ABA3 is a molybdenum cofactor sulfurase required for activation of aldehyde oxidase and xanthine dehydrogenase in Arabidopsis thaliana. J Biol Chem. 2001 Nov 2;276(44):40381-4. Epub 2001 Sep 11. Pubmed
66. Serine dehydratase-like
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q96GA7 
Gene: SDSL 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
67. P-selectin cytoplasmic tail-associated protein
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q96JQ3 
Gene: pcap 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
68. Hepatic peroxysomal alanine:glyoxylate aminotransferase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9BXA1 
Protein Sequence: FASTA
Gene Sequence: FASTA
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
69. Serine racemase
Pharmacological action: unknown
Actions: cofactor
Catalyzes the synthesis of D-serine from L-serine
Organism class: human
UniProt ID: Q9GZT4 
Gene: SRR 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Hashimoto A, Yoshikawa M: Effect of aminooxyacetic acid on extracellular level of D-serine in rat striatum: an in vivo microdialysis study. Eur J Pharmacol. 2005 Nov 21;525(1-3):91-3. Epub 2005 Nov 14. Pubmed
- Strisovsky K, Jiraskova J, Mikulova A, Rulisek L, Konvalinka J: Dual substrate and reaction specificity in mouse serine racemase: identification of high-affinity dicarboxylate substrate and inhibitors and analysis of the beta-eliminase activity. Biochemistry. 2005 Oct 4;44(39):13091-100. Pubmed
- Schell MJ: The N-methyl D-aspartate receptor glycine site and D-serine metabolism: an evolutionary perspective. Philos Trans R Soc Lond B Biol Sci. 2004 Jun 29;359(1446):943-64. Pubmed
- Strisovsky K, Jiraskova J, Barinka C, Majer P, Rojas C, Slusher BS, Konvalinka J: Mouse brain serine racemase catalyzes specific elimination of L-serine to pyruvate. FEBS Lett. 2003 Jan 30;535(1-3):44-8. Pubmed
- Uo T, Yoshimura T, Nishiyama T, Esaki N: Gene cloning, purification, and characterization of 2,3-diaminopropionate ammonia-lyase from Escherichia coli. Biosci Biotechnol Biochem. 2002 Dec;66(12):2639-44. Pubmed
70. O-phosphoseryl-tRNA(Sec) selenium transferase
Pharmacological action: unknown
Actions: cofactor
Converts O-phosphoseryl-tRNA(Sec) to selenocysteinyl- tRNA(Sec) required for selenoprotein biosynthesis
Organism class: human
UniProt ID: Q9HD40 
Gene: SEPSECS 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Xu XM, Carlson BA, Mix H, Zhang Y, Saira K, Glass RS, Berry MJ, Gladyshev VN, Hatfield DL: Biosynthesis of selenocysteine on its tRNA in eukaryotes. PLoS Biol. 2007 Jan;5(1):e4. Pubmed
- Kernebeck T, Lohse AW, Grotzinger J: A bioinformatical approach suggests the function of the autoimmune hepatitis target antigen soluble liver antigen/liver pancreas. Hepatology. 2001 Aug;34(2):230-3. Pubmed
71. Uncharacterized protein C20orf38
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9NUV7 
Gene: SPTLC3 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
72. Glutamic acid decarboxylase
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9UGI5 
Gene: GAD65 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Tong JC, Myers MA, Mackay IR, Zimmet PZ, Rowley MJ: The PEVKEK region of the pyridoxal phosphate binding domain of GAD65 expresses a dominant B cell epitope for type 1 diabetes sera. Ann N Y Acad Sci. 2002 Apr;958:182-9. Pubmed
- Burd L, Stenehjem A, Franceschini LA, Kerbeshian J: A 15-year follow-up of a boy with pyridoxine (vitamin B6)-dependent seizures with autism, breath holding, and severe mental retardation. J Child Neurol. 2000 Nov;15(11):763-5. Pubmed
- Myers MA, Davies JM, Tong JC, Whisstock J, Scealy M, Mackay IR, Rowley MJ: Conformational epitopes on the diabetes autoantigen GAD65 identified by peptide phage display and molecular modeling. J Immunol. 2000 Oct 1;165(7):3830-8. Pubmed
- Gospe SM Jr: Pyridoxine-dependent seizures: new genetic and biochemical clues to help with diagnosis and treatment. Curr Opin Neurol. 2006 Apr;19(2):148-53. Pubmed
- Hwang IK, Yoo KY, Kim do H, Lee BH, Kwon YG, Won MH: Time course of changes in pyridoxal 5’-phosphate (vitamin B6 active form) and its neuroprotection in experimental ischemic damage. Exp Neurol. 2007 Jul;206(1):114-25. Epub 2007 Apr 24. Pubmed
73. Alanine-glyoxylate aminotransferase homolog
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9UJX1 
Gene: TLH6 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Nishijima S, Sugaya K, Morozumi M, Hatano T, Ogawa Y: Hepatic alanine-glyoxylate aminotransferase activity and oxalate metabolism in vitamin B6 deficient rats. J Urol. 2003 Feb;169(2):683-6. Pubmed
- Gable K, Han G, Monaghan E, Bacikova D, Natarajan M, Williams R, Dunn TM: Mutations in the yeast LCB1 and LCB2 genes, including those corresponding to the hereditary sensory neuropathy type I mutations, dominantly inactivate serine palmitoyltransferase. J Biol Chem. 2002 Mar 22;277(12):10194-200. Epub 2002 Jan 7. Pubmed
- Nishijima S, Sugaya K, Morozumi M, Hatano T, Ogawa Y: Capillary electrophoresis assay of alanine:glyoxylate aminotransferase activity in rat liver. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Nov 15;780(1):13-9. Pubmed
- Nishijima S, Sugaya K, Hokama S, Oshiro Y, Uchida A, Morozumi M, Ogawa Y: Effect of vitamin B6 deficiency on glyoxylate metabolism in rats with or without glyoxylate overload. Biomed Res. 2006 Jun;27(3):93-8. Pubmed
- Danpure CJ, Lumb MJ, Birdsey GM, Zhang X: Alanine:glyoxylate aminotransferase peroxisome-to-mitochondrion mistargeting in human hereditary kidney stone disease. Biochim Biophys Acta. 2003 Apr 11;1647(1-2):70-5. Pubmed
74. Cysteine sulfinic acid decarboxylase-related protein 1
Pharmacological action: unknown
Actions: cofactor
Organism class: human
UniProt ID: Q9Y602 
Gene: CSAD 
Protein Sequence: FASTA
Gene Sequence: FASTA
SNPs: SNPJam Report
References:
- Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. Pubmed
- Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. Pubmed
- Skoldberg F, Rorsman F, Perheentupa J, Landin-Olsson M, Husebye ES, Gustafsson J, Kampe O: Analysis of antibody reactivity against cysteine sulfinic acid decarboxylase, a pyridoxal phosphate-dependent enzyme, in endocrine autoimmune disease. J Clin Endocrinol Metab. 2004 Apr;89(4):1636-40. Pubmed
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