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Identification
NamePyridoxal Phosphate
Accession NumberDB00114  (NUTR00045)
TypeSmall Molecule
GroupsNutraceutical
DescriptionThis is the active form of vitamin B6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (pyridoxamine). [PubChem]
Structure
Thumb
Synonyms
3-hydroxy-2-methyl-5-((phosphonooxy)methyl)-4-pyridinecarboxaldehyde
3-hydroxy-5-(hydroxymethyl)-2-methylisonicotinaldehyde 5-phosphate
Codecarboxylase
PLP
Pyridoxal 5-monophosphoric acid ester
Pyridoxal 5-phosphate
Pyridoxal 5'-phosphate
Pyridoxal P
Pyridoxal phosphate anhydrous
Pyridoxal-5P
Pyridoxal-P
External Identifiers Not Available
Approved Prescription ProductsNot Available
Approved Generic Prescription ProductsNot Available
Approved Over the Counter ProductsNot Available
Unapproved/Other Products Not Available
International Brands
NameCompany
BiosechsNot Available
HimitanNot Available
VitazechsNot Available
Brand mixturesNot Available
Salts
Name/CASStructureProperties
Pyridoxal phosphate hydrate
41468-25-1
Thumb
  • InChI Key: CEEQUQSGVRRXQI-UHFFFAOYSA-N
  • Monoisotopic Mass: 265.035138728
  • Average Mass: 265.158
DBSALT001801
Categories
UNIIF06SGE49M6
CAS number54-47-7
WeightAverage: 247.1419
Monoisotopic: 247.024573569
Chemical FormulaC8H10NO6P
InChI KeyInChIKey=NGVDGCNFYWLIFO-UHFFFAOYSA-N
InChI
InChI=1S/C8H10NO6P/c1-5-8(11)7(3-10)6(2-9-5)4-15-16(12,13)14/h2-3,11H,4H2,1H3,(H2,12,13,14)
IUPAC Name
[(4-formyl-5-hydroxy-6-methylpyridin-3-yl)methoxy]phosphonic acid
SMILES
CC1=NC=C(COP(O)(O)=O)C(C=O)=C1O
Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as pyridoxals and derivatives. These are compounds containing a pyridoxal moiety, which consists of a pyridine ring substituted at positions 2,3,4, and 5 by a methyl group, a hydroxyl group, a carbaldehyde group, and a hydroxymethyl group, respectively.
KingdomOrganic compounds
Super ClassOrganoheterocyclic compounds
ClassPyridines and derivatives
Sub ClassPyridine carboxaldehydes
Direct ParentPyridoxals and derivatives
Alternative Parents
Substituents
  • Pyridoxal
  • Methylpyridine
  • Hydroxypyridine
  • Monoalkyl phosphate
  • Aryl-aldehyde
  • Alkyl phosphate
  • Phosphoric acid ester
  • Organic phosphoric acid derivative
  • Organic phosphate
  • Heteroaromatic compound
  • Vinylogous acid
  • Azacycle
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aldehyde
  • Aromatic heteromonocyclic compound
Molecular FrameworkAromatic heteromonocyclic compounds
External Descriptors
Pharmacology
IndicationFor nutritional supplementation and for treating dietary shortage or imbalance.
PharmacodynamicsThe two major forms of vitamin B6 are pyridoxine and pyridoxamine. In the liver they are converted to pyridoxal phosphate (PLP) which is a cofactor in many reactions of amino acid metabolism. PLP also is necessary for the enzymatic reaction governing the release of glucose from glycogen. Pyroluria is one potential cause of vitamin B6 deficiency.
Mechanism of actionPyridoxal Phosphate is a coenzyme of many enzymatic reactions. It is the active form of vitamin B6 which comprises three natural organic compounds, pyridoxal, pyridoxamine and pyridoxine. Pyridoxal phosphate acts as a coenzyme in all transamination reactions, and in some decarboxylation and deamination reactions of amino acids. The aldehyde group of pyridoxal phosphate forms a Schiff-base linkage with the epsilon-amino group of a specific lysine group of the aminotransferase enzyme. The alpha-amino group of the amino acid substrate displaces the epsilon-amino group of the active-site lysine residue. The resulting aldimine becomes deprotonated to become a quinoid intermediate, which in turn accepts a proton at a different position to become a ketimine. The resulting ketimine is hydrolysed so that the amino group remains on the protein complex.
Related Articles
AbsorptionNot Available
Volume of distributionNot Available
Protein bindingNot Available
MetabolismNot Available
Route of eliminationNot Available
Half lifeNot Available
ClearanceNot Available
ToxicityNot Available
Affected organisms
  • Humans and other mammals
Pathways
PathwayCategorySMPDB ID
Tryptophan MetabolismMetabolicSMP00063
3-Methylglutaconic Aciduria Type IIIDiseaseSMP00140
HistidinemiaDiseaseSMP00191
HypoacetylaspartiaDiseaseSMP00192
Methylmalonic AciduriaDiseaseSMP00200
S-Adenosylhomocysteine (SAH) Hydrolase DeficiencyDiseaseSMP00214
Tyrosinemia Type IDiseaseSMP00218
Glycine N-methyltransferase DeficiencyDiseaseSMP00222
Non Ketotic HyperglycinemiaDiseaseSMP00223
Isovaleric AciduriaDiseaseSMP00238
Hereditary Coproporphyria (HCP)DiseaseSMP00342
Globoid Cell LeukodystrophyDiseaseSMP00348
Primary Hyperoxaluria Type IDiseaseSMP00352
ArgininemiaDiseaseSMP00357
Methylmalonate Semialdehyde Dehydrogenase DeficiencyDiseaseSMP00384
Disulfiram Action PathwayDrug actionSMP00429
Homocysteine DegradationMetabolicSMP00455
Carnitine SynthesisMetabolicSMP00465
Dimethylglycine Dehydrogenase DeficiencyDiseaseSMP00484
Tyrosine hydroxylase deficiencyDiseaseSMP00497
Malonyl-coa decarboxylase deficiencyDiseaseSMP00502
Isobutyryl-coa dehydrogenase deficiencyDiseaseSMP00523
Isovaleric acidemiaDiseaseSMP00524
Krabbe diseaseDiseaseSMP00526
Hyperlysinemia II or SaccharopinuriaDiseaseSMP00528
Glycogen synthetase deficiencyDiseaseSMP00552
Pyridoxine dependency with seizuresDiseaseSMP00571
sarcosine oncometabolite pathway DiseaseSMP02313
Beta-Alanine MetabolismMetabolicSMP00007
Catecholamine BiosynthesisMetabolicSMP00012
SNP Mediated EffectsNot Available
SNP Mediated Adverse Drug ReactionsNot Available
ADMET
Predicted ADMET features
PropertyValueProbability
Human Intestinal Absorption+0.5078
Blood Brain Barrier+0.8022
Caco-2 permeable-0.6476
P-glycoprotein substrateNon-substrate0.6324
P-glycoprotein inhibitor INon-inhibitor0.9035
P-glycoprotein inhibitor IINon-inhibitor0.9694
Renal organic cation transporterNon-inhibitor0.882
CYP450 2C9 substrateNon-substrate0.6828
CYP450 2D6 substrateNon-substrate0.7978
CYP450 3A4 substrateNon-substrate0.5915
CYP450 1A2 substrateNon-inhibitor0.8704
CYP450 2C9 inhibitorNon-inhibitor0.9041
CYP450 2D6 inhibitorNon-inhibitor0.901
CYP450 2C19 inhibitorNon-inhibitor0.8772
CYP450 3A4 inhibitorNon-inhibitor0.9308
CYP450 inhibitory promiscuityLow CYP Inhibitory Promiscuity0.9352
Ames testNon AMES toxic0.6624
CarcinogenicityNon-carcinogens0.8948
BiodegradationReady biodegradable0.5443
Rat acute toxicity1.6531 LD50, mol/kg Not applicable
hERG inhibition (predictor I)Weak inhibitor0.8729
hERG inhibition (predictor II)Non-inhibitor0.9128
ADMET data is predicted using admetSAR, a free tool for evaluating chemical ADMET properties. (23092397 )
Pharmacoeconomics
ManufacturersNot Available
Packagers
Dosage formsNot Available
PricesNot Available
PatentsNot Available
Properties
StateSolid
Experimental Properties
PropertyValueSource
melting point255 °CPhysProp
water solubilityAppreciableNot Available
logP-1.2Not Available
Predicted Properties
PropertyValueSource
Water Solubility5.7 mg/mLALOGPS
logP-0.55ALOGPS
logP-2.1ChemAxon
logS-1.6ALOGPS
pKa (Strongest Acidic)1.68ChemAxon
pKa (Strongest Basic)4.11ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count6ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area116.95 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity54.75 m3·mol-1ChemAxon
Polarizability20.9 Å3ChemAxon
Number of Rings1ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Mass Spec (NIST)Not Available
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 3 TMS)splash10-0gb9-2690000000-c9bacb7e657461e28407View in MoNA
GC-MSGC-MS Spectrum - GC-MSNot Available
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0f6t-0690000000-4aa57f3f28f0bdef9dcfView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0fxx-8900000000-ee972556340c61650528View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-014l-9100000000-3cc29fb51820adecd59cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-0002-0090000000-9b86e80a9dd0de14ab59View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-03dj-9800000000-9313aa9cbf19bc6311c5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0002-9500000000-7ce120a58879e2214ce5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-004j-9200000000-7e6bd8c298613e59fb5dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-004i-9000000000-4c05178b1645bf01f3faView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0udi-3920000000-6c5e6106f5658d1d6c50View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0udi-3920000000-f96097815e6922356131View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-002b-9000000000-2c4e243699a95e3a0f88View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-002b-9000000000-8aa111485891af9f0d02View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0f6t-1790000000-1dcb07cd1110c71f7005View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0udi-0900000000-ca6aab7f31a36863a417View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0udi-9800000000-053802b9c0533d7943eaView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0002-9080000000-12ca63d34d39d59f6b42View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-004i-9000000000-144099ec201adbbc4684View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-004i-9000000000-70a9559d65e78c488e7eView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
References
Synthesis Reference

Robert C. Siegel, “Synthesis of cross-links in the helical domain of collagen using pyridoxal 5-phosphate and copper or iron.” U.S. Patent US4544638, issued June, 1981.

US4544638
General ReferencesNot Available
External Links
ATC CodesA11HA06
AHFS CodesNot Available
PDB Entries
FDA labelNot Available
MSDSDownload (36.1 KB)
Interactions
Drug InteractionsNot Available
Food InteractionsNot Available

Targets

Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.
Gene Name:
AGXT2
Uniprot ID:
Q9BYV1
Molecular Weight:
57155.905 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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. 1995 Apr;117(4):856-62. [PubMed:7592550 ]
  4. 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:6703688 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the production of GABA.
Gene Name:
GAD1
Uniprot ID:
Q99259
Molecular Weight:
66896.065 Da
References
  1. 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:15381280 ]
  2. 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:11488610 ]
  3. 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:15686475 ]
  4. 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:12535612 ]
  5. 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:12067524 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Ubiquitin protein ligase binding
Specific Function:
Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (PubMed:23981774, PubMed:20506325, PubMed:23974653). Also involved in the...
Gene Name:
CBS
Uniprot ID:
P35520
Molecular Weight:
60586.05 Da
References
  1. 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:11948191 ]
  2. 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:12173932 ]
  3. 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:12644499 ]
  4. 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:15196993 ]
  5. 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:15365998 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the cleavage of L-kynurenine (L-Kyn) and L-3-hydroxykynurenine (L-3OHKyn) into anthranilic acid (AA) and 3-hydroxyanthranilic acid (3-OHAA), respectively. Has a preference for the L-3-hydroxy form. Also has cysteine-conjugate-beta-lyase activity.
Gene Name:
KYNU
Uniprot ID:
Q16719
Molecular Weight:
52351.14 Da
References
  1. 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:14756555 ]
  2. 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:12204580 ]
  3. 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:17300176 ]
  4. 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:11985583 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Serine binding
Specific Function:
Interconversion of serine and glycine.
Gene Name:
SHMT1
Uniprot ID:
P34896
Molecular Weight:
53082.18 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:7526359 ]
  4. 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:9305893 ]
  5. 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:3143355 ]
  6. 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:10592235 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor.
Gene Name:
NFS1
Uniprot ID:
Q9Y697
Molecular Weight:
50195.21 Da
References
  1. 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:12119030 ]
  2. 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:17469805 ]
  3. 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:16310786 ]
  4. 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:12860127 ]
  5. 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:12196163 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
activator
General Function:
Pyridoxal phosphate binding
Specific Function:
Biosynthesis of L-glutamate from L-aspartate or L-cysteine. Important regulator of levels of glutamate, the major excitatory neurotransmitter of the vertebrate central nervous system. Acts as a scavenger of glutamate in brain neuroprotection. The aspartate aminotransferase activity is involved in hepatic glucose synthesis during development and in adipocyte glyceroneogenesis. Using L-cysteine a...
Gene Name:
GOT1
Uniprot ID:
P17174
Molecular Weight:
46247.14 Da
References
  1. 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:11340119 ]
  2. 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:11888303 ]
  3. 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:12167474 ]
  4. 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:16925884 ]
  5. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
OAT
Uniprot ID:
P04181
Molecular Weight:
48534.39 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:10592235 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Protein homodimerization activity
Specific Function:
Key enzyme of polyamine biosynthesis that converts ornithine into putrescine, which is the precursor for the polyamines, spermidine and spermine.
Gene Name:
ODC1
Uniprot ID:
P11926
Molecular Weight:
51147.73 Da
References
  1. 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:12672797 ]
  2. 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:12627959 ]
  3. 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:15476392 ]
  4. Khomutov AR: Inhibition of enzymes of polyamine biosynthesis by substrate-like O-substituted hydroxylamines. Biochemistry (Mosc). 2002 Oct;67(10):1159-67. [PubMed:12460114 ]
  5. 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:11683631 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Transaminase with broad substrate specificity. Has transaminase activity towards aminoadipate, kynurenine, methionine and glutamate. Shows activity also towards tryptophan, aspartate and hydroxykynurenine. Accepts a variety of oxo-acids as amino-group acceptors, with a preference for 2-oxoglutarate, 2-oxocaproic acid, phenylpyruvate and alpha-oxo-gamma-methiol butyric acid. Can also use glyoxyl...
Gene Name:
AADAT
Uniprot ID:
Q8N5Z0
Molecular Weight:
47351.17 Da
References
  1. 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:12126930 ]
  2. 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:15016471 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
inhibitor
General Function:
Succinate-semialdehyde dehydrogenase binding
Specific Function:
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.
Gene Name:
ABAT
Uniprot ID:
P80404
Molecular Weight:
56438.405 Da
References
  1. 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:14534310 ]
  2. 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:17531224 ]
  3. 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:14567543 ]
  4. 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:16096275 ]
  5. 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:15723541 ]
  6. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxamine-phosphate oxidase activity
Specific Function:
Catalyzes the oxidation of either pyridoxine 5'-phosphate (PNP) or pyridoxamine 5'-phosphate (PMP) into pyridoxal 5'-phosphate (PLP).
Gene Name:
PNPO
Uniprot ID:
Q9NVS9
Molecular Weight:
29987.79 Da
References
  1. 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:16538088 ]
  2. 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:16207494 ]
  3. Pearl PL, Hartka TR, Taylor J: Diagnosis and treatment of neurotransmitter disorders. Curr Treat Options Neurol. 2006 Nov;8(6):441-50. [PubMed:17032564 ]
  4. 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:17216302 ]
  5. 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:15772097 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Sphinganine-1-phosphate aldolase activity
Specific Function:
Cleaves phosphorylated sphingoid bases (PSBs), such as sphingosine-1-phosphate, into fatty aldehydes and phosphoethanolamine. Elevates stress-induced ceramide production and apoptosis.
Gene Name:
SGPL1
Uniprot ID:
O95470
Molecular Weight:
63523.265 Da
References
  1. 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:15522238 ]
  2. 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:11377878 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Transaminase involved in tyrosine breakdown. Converts tyrosine to p-hydroxyphenylpyruvate. Can catalyze the reverse reaction, using glutamic acid, with 2-oxoglutarate as cosubstrate (in vitro). Has much lower affinity and transaminase activity towards phenylalanine.
Gene Name:
TAT
Uniprot ID:
P17735
Molecular Weight:
50398.895 Da
References
  1. 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:16570355 ]
  2. 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:16902803 ]
  3. 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:16466641 ]
  4. 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:12441383 ]
  5. 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:12798391 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine 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.
Gene Name:
CCBL1
Uniprot ID:
Q16773
Molecular Weight:
47874.765 Da
References
  1. 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:12167474 ]
  2. 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:12850267 ]
  3. 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:16846239 ]
  4. 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:12798391 ]
  5. Cooper AJ, Pinto JT: Cysteine S-conjugate beta-lyases. Amino Acids. 2006 Feb;30(1):1-15. Epub 2006 Feb 6. [PubMed:16463021 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Threonine synthase activity
Specific Function:
Not Available
Gene Name:
THNSL1
Uniprot ID:
Q8IYQ7
Molecular Weight:
83069.54 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Vitamin binding
Specific Function:
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.
Gene Name:
PYGL
Uniprot ID:
P06737
Molecular Weight:
97147.82 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:17705025 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Serine c-palmitoyltransferase activity
Specific Function:
Serine palmitoyltransferase (SPT). The heterodimer formed with LCB1/SPTLC1 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SPTSSA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC2-SPTSSB complex displays a preference for C18-CoA substrate.
Gene Name:
SPTLC2
Uniprot ID:
O15270
Molecular Weight:
62923.765 Da
References
  1. Hanada K: Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. Biochim Biophys Acta. 2003 Jun 10;1632(1-3):16-30. [PubMed:12782147 ]
  2. 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:11726713 ]
  3. 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:11781309 ]
  4. 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:11279212 ]
  5. 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:11171191 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Sulfinoalanine decarboxylase activity
Specific Function:
Not Available
Gene Name:
CSAD
Uniprot ID:
Q9Y600
Molecular Weight:
55022.79 Da
References
  1. 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:15070923 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the biosynthesis of histamine from histidine.
Gene Name:
HDC
Uniprot ID:
P19113
Molecular Weight:
74139.825 Da
References
  1. 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:12679420 ]
  2. 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:16547811 ]
  3. 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:16790022 ]
  4. 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:15070923 ]
  5. 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:11980912 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Putrescine transmembrane transporter activity
Specific Function:
Antizyme inhibitor protein that positively regulates ornithine decarboxylase (ODC) activity and polyamine uptake by counteracting the negative effect of antizymes OAZ1, OAZ2 and OAZ3 on ODC1 activity (PubMed:17900240). Inhibits antizyme-dependent ODC1 degradation by binding to antizymes. Releases ODC1 from its inactive complex with antizymes, leading to formation of the catalytically active ODC...
Gene Name:
AZIN2
Uniprot ID:
Q96A70
Molecular Weight:
49979.185 Da
References
  1. 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:11980912 ]
  2. 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:15583399 ]
  3. 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:16997906 ]
  4. Arena ME, Manca de Nadra MC: Biogenic amine production by Lactobacillus. J Appl Microbiol. 2001 Feb;90(2):158-62. [PubMed:11168717 ]
  5. 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:17305368 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
SDS
Uniprot ID:
P20132
Molecular Weight:
34625.105 Da
References
  1. 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:15689518 ]
  2. 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:14596599 ]
  3. 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:14646100 ]
  4. 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:16580895 ]
  5. 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:15155761 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
GCAT
Uniprot ID:
O75600
Molecular Weight:
45284.6 Da
References
  1. 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:11318637 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Lyase activity
Specific Function:
The glycine cleavage system catalyzes the degradation of glycine. The P protein (GLDC) 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 (GCSH).
Gene Name:
GLDC
Uniprot ID:
P23378
Molecular Weight:
112728.805 Da
References
  1. 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:15791207 ]
  2. 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:16228305 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate. Participates in cellular nitrogen metabolism and also in liver gluconeogenesis starting with precursors transported from skeletal muscles (By similarity).
Gene Name:
GPT
Uniprot ID:
P24298
Molecular Weight:
54636.415 Da
References
  1. 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. 2003 Nov;134(5):731-8. [PubMed:14688239 ]
  2. 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:12537583 ]
  3. 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:16179747 ]
  4. 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:15347780 ]
  5. 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:11330835 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
O-phospho-l-serine:2-oxoglutarate aminotransferase activity
Specific Function:
Catalyzes the reversible conversion of 3-phosphohydroxypyruvate to phosphoserine and of 3-hydroxy-2-oxo-4-phosphonooxybutanoate to phosphohydroxythreonine.
Gene Name:
PSAT1
Uniprot ID:
Q9Y617
Molecular Weight:
40422.355 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:8695645 ]
  4. 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:10024454 ]
  5. 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:16532449 ]
  6. 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:10592235 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
ALAS1
Uniprot ID:
P13196
Molecular Weight:
70580.325 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:7275938 ]
  4. 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:10577279 ]
  5. 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:12686158 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Not Available
Gene Name:
AGXT
Uniprot ID:
P21549
Molecular Weight:
43009.535 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:16971151 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphatase activity
Specific Function:
Protein serine phosphatase that dephosphorylates 'Ser-3' in cofilin and probably also dephosphorylates phospho-serine residues in DSTN. Regulates cofilin-dependent actin cytoskeleton reorganization. Required for normal progress through mitosis and normal cytokinesis. Does not dephosphorylate phospho-threonines in LIMK1. Does not dephosphorylate peptides containing phospho-tyrosine. Pyridoxal ph...
Gene Name:
PDXP
Uniprot ID:
Q96GD0
Molecular Weight:
31697.735 Da
References
  1. 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:14522954 ]
  2. 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:11000053 ]
  3. 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:17531224 ]
  4. 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:16336786 ]
  5. 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:16336194 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Serine c-palmitoyltransferase activity
Specific Function:
Serine palmitoyltransferase (SPT). The heterodimer formed with SPTLC2 or SPTLC3 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC2-SPTSSA complex shows a strong preference for C16-CoA substrate, while the SPTLC1-SPTLC3-SPTSSA isozyme uses both C14-CoA and C16-CoA as substrates, with a slight pre...
Gene Name:
SPTLC1
Uniprot ID:
O15269
Molecular Weight:
52743.41 Da
References
  1. Hanada K: Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. Biochim Biophys Acta. 2003 Jun 10;1632(1-3):16-30. [PubMed:12782147 ]
  2. 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:11781309 ]
  3. 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:11279212 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling mol...
Gene Name:
CTH
Uniprot ID:
P32929
Molecular Weight:
44507.64 Da
References
  1. 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:15184188 ]
  2. 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:15823094 ]
  3. Lowicka E, Beltowski J: Hydrogen sulfide (H2S) - the third gas of interest for pharmacologists. Pharmacol Rep. 2007 Jan-Feb;59(1):4-24. [PubMed:17377202 ]
  4. 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:16857832 ]
  5. 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:11578145 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
L-valine transaminase activity
Specific Function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular Weight:
42965.815 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
L-valine transaminase activity
Specific Function:
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.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular Weight:
44287.445 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
PROSC
Uniprot ID:
O94903
Molecular Weight:
30343.7 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Microtubule binding
Specific Function:
Folate-dependent enzyme, that displays both transferase and deaminase activity. Serves to channel one-carbon units from formiminoglutamate to the folate pool.Binds and promotes bundling of vimentin filaments originating from the Golgi.
Gene Name:
FTCD
Uniprot ID:
O95954
Molecular Weight:
58925.93 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). Plays a key role in amino acid metabolism. Important for metabolite exchange between mitochondria and cytosol. Facilitates cellular uptake of long-chain free fatty acids.
Gene Name:
GOT2
Uniprot ID:
P00505
Molecular Weight:
47517.285 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:236311 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
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.
Gene Name:
PYGB
Uniprot ID:
P11216
Molecular Weight:
96695.18 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
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.
Gene Name:
PYGM
Uniprot ID:
P11217
Molecular Weight:
97091.265 Da
References
  1. 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:12460107 ]
  2. 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:11052666 ]
  3. 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:11228757 ]
  4. 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:11508701 ]
  5. 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:12217700 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the decarboxylation of L-3,4-dihydroxyphenylalanine (DOPA) to dopamine, L-5-hydroxytryptophan to serotonin and L-tryptophan to tryptamine.
Gene Name:
DDC
Uniprot ID:
P20711
Molecular Weight:
53925.815 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:6983619 ]
  4. 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:7567987 ]
  5. 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:11085948 ]
  6. 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. doi: 10.1111/j.1471-4159.2010.06742.x. Epub 2010 Apr 9. [PubMed:20403077 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
GIG18
Uniprot ID:
Q2TU84
Molecular Weight:
46319.2 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Amino acid transport and metabolism
Specific Function:
Not Available
Gene Name:
GAD1
Uniprot ID:
Q49AK1
Molecular Weight:
47440.0 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:16075246 ]
  4. 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:8499653 ]
  5. 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:10978228 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Interconversion of serine and glycine.
Gene Name:
Not Available
Uniprot ID:
Q53ET4
Molecular Weight:
55973.345 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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. 2008 Mar;52(3):379-85. [PubMed:17918169 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Lyase activity
Specific Function:
Not Available
Gene Name:
Not Available
Uniprot ID:
Q59FK2
Molecular Weight:
25484.43 Da
References
  1. 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:15561708 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
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.
Gene Name:
Not Available
Uniprot ID:
Q59GM9
Molecular Weight:
98828.62 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Not Available
Gene Name:
Not Available
Uniprot ID:
Q59HE2
Molecular Weight:
30736.21 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
ALAS1
Uniprot ID:
Q5JAM2
Molecular Weight:
70580.325 Da
References
  1. 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:12699240 ]
  2. 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:15251194 ]
  3. 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:12191993 ]
  4. 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:17469798 ]
  5. 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:15840827 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
GAD2
Uniprot ID:
Q5VZ30
Molecular Weight:
65410.77 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:16075246 ]
  4. 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:8499653 ]
  5. 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:10978228 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
DDC
Uniprot ID:
Q6IBS8
Molecular Weight:
53879.725 Da
References
  1. 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;137B(1):1-4. [PubMed:15965967 ]
  2. 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:11085948 ]
  3. 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:11709201 ]
  4. 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:12679420 ]
  5. 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:15070923 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
PDXDC1
Uniprot ID:
Q6P996
Molecular Weight:
86706.135 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine 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 (By similarity). Has transaminase activity towards L-kynurenine, tryptophan, phenylalanine, serine, cysteine, methionine, histidine, glutamin...
Gene Name:
CCBL2
Uniprot ID:
Q6YP21
Molecular Weight:
51399.855 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Sulfinoalanine decarboxylase activity
Specific Function:
May catalyze the decarboxylation of aspartate, cysteine sulfinic acid, and cysteic acid to beta-alanine, hypotaurine and taurine, respectively. Does not exhibit any decarboxylation activity toward glutamate.
Gene Name:
GADL1
Uniprot ID:
Q6ZQY3
Molecular Weight:
59245.95 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transferase activity
Specific Function:
Catalyzes the decomposition of L-selenocysteine to L-alanine and elemental selenium.
Gene Name:
SCLY
Uniprot ID:
Q96I15
Molecular Weight:
48148.45 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:10592235 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Not Available
Specific Function:
May be involved in the maintenance of osteochondroprogenitor cells pool.
Gene Name:
IGSF10
Uniprot ID:
Q6WRI0
Molecular Weight:
290835.42 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:10592235 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Catalyzes the pyridoxal-phosphate-dependent breakdown of 5-phosphohydroxy-L-lysine, converting it to ammonia, inorganic phosphate and 2-aminoadipate semialdehyde.
Gene Name:
PHYKPL
Uniprot ID:
Q8IUZ5
Molecular Weight:
49710.245 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
GAD1
Uniprot ID:
Q8IVA8
Molecular Weight:
66916.045 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:16075246 ]
  4. 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:8499653 ]
  5. 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:10978228 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Required to prevent uracil accumulation in mtDNA. Interconversion of serine and glycine. Associates with mitochondrial DNA.
Gene Name:
SHMT2
Uniprot ID:
P34897
Molecular Weight:
55992.385 Da
References
  1. 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:15865438 ]
  2. 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:11877399 ]
  3. 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:17482557 ]
  4. 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:17651438 ]
  5. 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:16980404 ]
  6. 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:7526359 ]
  7. 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:9305893 ]
  8. 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:3143355 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
ALAS2
Uniprot ID:
P22557
Molecular Weight:
64632.86 Da
References
  1. 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:12686158 ]
  2. 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:15251194 ]
  3. 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:14724775 ]
  4. Clayton PT: B6-responsive disorders: a model of vitamin dependency. J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):317-26. [PubMed:16763894 ]
  5. 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:16121195 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate.
Gene Name:
GPT2
Uniprot ID:
Q8TD30
Molecular Weight:
57903.11 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
  3. 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:241701 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transferase activity
Specific Function:
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. In vitro, the C-terminal domain is able to reduce N-hydroxylated prodrugs, such as benzamidoxime.
Gene Name:
MOCOS
Uniprot ID:
Q96EN8
Molecular Weight:
98118.965 Da
References
  1. 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:15561708 ]
  2. Mendel RR, Bittner F: Cell biology of molybdenum. Biochim Biophys Acta. 2006 Jul;1763(7):621-35. Epub 2006 May 12. [PubMed:16784786 ]
  3. 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:11886751 ]
  4. 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:11553608 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Has low serine dehydratase and threonine dehydratase activity.
Gene Name:
SDSL
Uniprot ID:
Q96GA7
Molecular Weight:
34674.01 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
pcap
Uniprot ID:
Q96JQ3
Molecular Weight:
29222.465 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Not Available
Gene Name:
Not Available
Uniprot ID:
Q9BXA1
Molecular Weight:
39774.51 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Threonine racemase activity
Specific Function:
Catalyzes the synthesis of D-serine from L-serine. D-serine is a key coagonist with glutamate at NMDA receptors. Has dehydratase activity towards both L-serine and D-serine.
Gene Name:
SRR
Uniprot ID:
Q9GZT4
Molecular Weight:
36565.905 Da
References
  1. 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:16289454 ]
  2. 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:16185077 ]
  3. 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:15306409 ]
  4. 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:12560076 ]
  5. 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:12596860 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Trna binding
Specific Function:
Converts O-phosphoseryl-tRNA(Sec) to selenocysteinyl-tRNA(Sec) required for selenoprotein biosynthesis.
Gene Name:
SEPSECS
Uniprot ID:
Q9HD40
Molecular Weight:
55725.69 Da
References
  1. 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:17194211 ]
  2. 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:11481605 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Serine c-palmitoyltransferase activity
Specific Function:
Serine palmitoyltransferase (SPT). The heterodimer formed with LCB1/SPTLC1 constitutes the catalytic core. The composition of the serine palmitoyltransferase (SPT) complex determines the substrate preference. The SPTLC1-SPTLC3-SPTSSA isozyme uses both C14-CoA and C16-CoA as substrates, while the SPTLC1-SPTLC3-SPTSSB has the ability to use a broader range of acyl-CoAs without apparent preference.
Gene Name:
SPTLC3
Uniprot ID:
Q9NUV7
Molecular Weight:
62049.035 Da
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
  2. 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:17016423 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Pyridoxal phosphate binding
Specific Function:
Not Available
Gene Name:
GAD65
Uniprot ID:
Q9UGI5
Molecular Weight:
47343.69 Da
References
  1. 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:12021103 ]
  2. 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:11108513 ]
  3. 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:11034389 ]
  4. 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:16538088 ]
  5. 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:17531224 ]
Kind
Protein
Organism
Human
Pharmacological action
unknown
Actions
cofactor
General Function:
Transaminase activity
Specific Function:
Not Available
Gene Name:
TLH6
Uniprot ID:
Q9UJX1
Molecular Weight:
28429.215 Da
References
  1. 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:12544342 ]
  2. 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:11781309 ]
  3. 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:12383475 ]
  4. 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:16847354 ]
  5. 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:12686111 ]
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Drug created on June 13, 2005 07:24 / Updated on August 17, 2016 12:23