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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-10-29 21:50:12 UTC
HMDB IDHMDB00208
Secondary Accession Numbers
  • HMDB02812
Metabolite Identification
Common NameOxoglutaric acid
DescriptionAlpha-ketoglutaric acid is an important biological compound and is a key intermediate in the Krebs cycle. Alpha-ketoglutaric acid occurs naturally within cells. One of its functions is to combine with ammonia to form glutamic acid and then glutamine. Another function is to combine with nitrogen released in the cell, therefore preventing nitrogen overload. (wikipedia).
Structure
Thumb
Synonyms
  1. 2-Ketoglutarate
  2. 2-Ketoglutaric acid
  3. 2-Oxo-1,5-pentanedioate
  4. 2-Oxo-1,5-pentanedioic acid
  5. 2-Oxoglutarate
  6. 2-Oxoglutaric acid
  7. 2-Oxopentanedioate
  8. 2-Oxopentanedioic acid
  9. alpha-Ketoglutaric acid
  10. Oxoglutarate
Chemical FormulaC5H6O5
Average Molecular Weight146.0981
Monoisotopic Molecular Weight146.021523302
IUPAC Name2-oxopentanedioic acid
Traditional Name2-oxo-glutaric acid
CAS Registry Number328-50-7
SMILES
OC(=O)CCC(=O)C(O)=O
InChI Identifier
InChI=1S/C5H6O5/c6-3(5(9)10)1-2-4(7)8/h1-2H2,(H,7,8)(H,9,10)
InChI KeyKPGXRSRHYNQIFN-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassOrganic Acids and Derivatives
ClassKeto-Acids and Derivatives
Sub ClassGamma Keto-Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Fatty Acids and Conjugates
  • oxo dicarboxylic acid(ChEBI)
Substituents
  • Alpha Keto Acid
  • Carboxylic Acid
  • Dicarboxylic Acid Derivative
  • Ketone
  • Short Chain Keto Acid
Direct ParentGamma Keto-Acids and Derivatives
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Microbial
Biofunction
  • Component of Alanine and aspartate metabolism
  • Component of Arginine and proline metabolism
  • Component of Cysteine metabolism
  • Component of D-Glutamine and D-glutamate metabolism
  • Component of Glutamate metabolism
  • Component of Glutathione metabolism
  • Component of Glycine, serine and threonine metabolism
  • Component of Lysine biosynthesis
  • Component of Nitrogen metabolism
  • Component of Novobiocin biosynthesis
  • Component of Pantothenate and CoA biosynthesis
  • Component of Phenylalanine metabolism
  • Component of Phenylalanine, tyrosine and tryptophan biosynthesis
  • Component of Tryptophan metabolism
  • Component of Tyrosine metabolism
  • Component of Valine, leucine and isoleucine biosynthesis
  • Component of Vitamin B6 metabolism
ApplicationNot Available
Cellular locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point115.5 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility541.5 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility53.1ALOGPS
logP-0.6ALOGPS
logP-0.11ChemAxon
logS-0.44ALOGPS
pKa (Strongest Acidic)2.66ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area91.67 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity28.88 m3·mol-1ChemAxon
Polarizability12.17 Å3ChemAxon
Spectra
SpectraGC-MSMS/MSLC-MSMS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Extracellular
  • Mitochondria
  • Endoplasmic reticulum
  • Peroxisome
Biofluid Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Alanine MetabolismSMP00055map00250
Ammonia RecyclingSMP00009map00910
Carnitine SynthesisSMP00465Not Available
Citric Acid CycleSMP00057map00020
GluconeogenesisSMP00128map00010
Glucose-Alanine CycleSMP00127Not Available
Glutamate MetabolismSMP00072map00250
Glycine and Serine MetabolismSMP00004map00260
Malate-Aspartate ShuttleSMP00129Not Available
Phytanic Acid Peroxisomal OxidationSMP00450Not Available
Urea CycleSMP00059map00330
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified7.0 (0.0-23.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.6 +/- 2.6 uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified9.3 +/- 2.3 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified8.9 +/- 2.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified20.7 +/- 2.5 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
details
Cellular CytoplasmDetected and Quantified320 (290-350) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5 +/- 4 uMNot SpecifiedBothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified2.1 +/- 0.7 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified8.9 +/- 3.4 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
Cerebrospinal Fluid (CSF)Detected and Quantified2.0 +/- 2.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.80 (3.40-6.20) uMChildren (1-13 years old)BothNormal details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified5.27 +/- 3.61 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot ApplicableAdult (>18 years old)Male
Normal
details
SalivaDetected and Quantified1.53 +/- 1.18 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.64 +/- 3.47 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.65 +/- 1.07 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.65 +/- 1.07 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified3.78 +/- 2.33 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified<150 umol/mmol creatinineAdult (>18 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.87 (0.18-14.3) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified2.98 (0.42-18.3) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified23.6 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified8.90-17.12 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected and Quantified4.2 +/- 1.97 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified24 (4-74) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified150.5 (10.0-836.3) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified236.9 (8.0-631.4) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified21.4 (4.9-123.8) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified25.6 (10.2-146.0) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified4.8 (2.0-17.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.2-8.2 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified43.46 +/- 53.46 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified101.0 (50.0-152.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified> 0.658 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified3.4 (1.4-12.5) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cellular CytoplasmDetected and Quantified150 (144-156) uMAdult (>18 years old)BothAnoxia details
UrineDetected and Quantified200.0 (150.0-250.0) umol/mmol creatinineChildren (1-13 years old)BothFumaric Aciduria details
UrineDetected and Quantified875.0 (150.0-1600.0) umol/mmol creatinineChildren (1-13 years old)Both2-Ketoglutarate Dehydrogenase Complex Deficiency details
Associated Disorders and Diseases
Disease References
2-Ketoglutarate dehydrogenase complex deficiency
  1. MetaGene
  2. MetaGene
Anoxia
  1. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. Pubmed: 8579834
Fumarase deficiency
  1. MetaGene
Associated OMIM IDs
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 606812 (Fumarase deficiency)
DrugBank IDDB02926
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB003361
KNApSAcK IDC00000769
Chemspider ID50
KEGG Compound IDC00026
BioCyc ID2-KETOGLUTARATE
BiGG ID33565
Wikipedia LinkOxoglutarate
NuGOwiki LinkHMDB00208
Metagene LinkHMDB00208
METLIN ID5218
PubChem Compound51
PDB IDAKG
ChEBI ID30915
References
Synthesis ReferenceTanaka, Katsunobu; kimura, Kazu; Yamaguchi, Ken. Fermentative production of a-oxoglutaric acid. U.S. (1973), 4 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Nicholson JK, Foxall PJ, Spraul M, Farrant RD, Lindon JC: 750 MHz 1H and 1H-13C NMR spectroscopy of human blood plasma. Anal Chem. 1995 Mar 1;67(5):793-811. Pubmed: 7762816
  2. Sweatman BC, Farrant RD, Holmes E, Ghauri FY, Nicholson JK, Lindon JC: 600 MHz 1H-NMR spectroscopy of human cerebrospinal fluid: effects of sample manipulation and assignment of resonances. J Pharm Biomed Anal. 1993 Aug;11(8):651-64. Pubmed: 8257730
  3. Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7. Pubmed: 9869358
  4. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. Pubmed: 8087979
  5. Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. Pubmed: 12829005
  6. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. Pubmed: 8579834
  7. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. Pubmed: 6321058
  8. Stromme JH, Borud O, Moe PJ: Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase. Pediatr Res. 1976 Jan;10(1):62-6. Pubmed: 813176
  9. Rocchiccioli F, Leroux JP, Cartier PH: Microdetermination of 2-ketoglutaric acid in plasma and cerebrospinal fluid by capillary gas chromatography mass spectrometry; application to pediatrics. Biomed Mass Spectrom. 1984 Jan;11(1):24-8. Pubmed: 6704500
  10. Barbas C, Garcia A, de Miguel L, Simo C: Evaluation of filter paper collection of urine samples for detection and measurement of organic acidurias by capillary electrophoresis. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Nov 15;780(1):73-82. Pubmed: 12383482
  11. Kitaura J, Miki Y, Kato H, Sakakihara Y, Yanagisawa M: Hyperinsulinaemic hypoglycaemia associated with persistent hyperammonaemia. Eur J Pediatr. 1999 May;158(5):410-3. Pubmed: 10333126
  12. Kelley RI: Octenylsuccinic aciduria in children fed protein-hydrolysate formulas containing modified cornstarch. Pediatr Res. 1991 Dec;30(6):564-9. Pubmed: 1805153
  13. Garcia A, Barbas C, Aguilar R, Castro M: Capillary electrophoresis for rapid profiling of organic acidurias. Clin Chem. 1998 Sep;44(9):1905-11. Pubmed: 9732975

Only showing the first 50 proteins. There are 68 proteins in total.

Enzymes

General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH1
Uniprot ID:
O75874
Molecular weight:
46659.005
Reactions
Isocitric acid + NADP → Oxoglutaric acid + CO(2) + NADPHdetails
Isocitric acid + NADP → Oxoglutaric acid + Carbon dioxide + NADPH + Hydrogen Iondetails
Oxalosuccinic acid → Oxoglutaric acid + Carbon dioxidedetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
OGDH
Uniprot ID:
Q02218
Molecular weight:
48179.59
Reactions
Oxoglutaric acid + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine → [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)details
Oxoglutaric acid + Thiamine pyrophosphate → 3-Carboxy-1-hydroxypropylthiamine diphosphate + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity).
Gene Name:
GLUD1
Uniprot ID:
P00367
Molecular weight:
61397.315
Reactions
L-Glutamic acid + Water + NAD(P)(+) → Oxoglutaric acid + Ammonia + NAD(P)Hdetails
L-Glutamic acid + NAD + Water → Oxoglutaric acid + Ammonia + NADH + Hydrogen Iondetails
L-Glutamic acid + NADP + Water → Oxoglutaric acid + Ammonia + NADPH + Hydrogen Iondetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Plays a key role in amino acid metabolism (By similarity).
Gene Name:
GOT1
Uniprot ID:
P17174
Molecular weight:
46247.14
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + L-Glutamic aciddetails
3-Sulfinoalanine + Oxoglutaric acid → B-Sulfinyl pyruvate + L-Glutamic aciddetails
4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + L-Glutamic aciddetails
General function:
Involved in transporter activity
Specific function:
Catalyzes the transport of 2-oxoglutarate across the inner mitochondrial membrane in an electroneutral exchange for malate or other dicarboxylic acids, and plays an important role in several metabolic processes, including the malate-aspartate shuttle, the oxoglutarate/isocitrate shuttle, in gluconeogenesis from lactate, and in nitrogen metabolism.
Gene Name:
SLC25A11
Uniprot ID:
Q02978
Molecular weight:
34061.405
General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH3A
Uniprot ID:
P50213
Molecular weight:
39591.365
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADHdetails
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH3G
Uniprot ID:
P51553
Molecular weight:
42793.97
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADHdetails
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in 2-hydroxyglutarate dehydrogenase activity
Specific function:
Not Available
Gene Name:
L2HGDH
Uniprot ID:
Q9H9P8
Molecular weight:
50315.58
Reactions
L-2-Hydroxyglutaric acid + acceptor → Oxoglutaric acid + reduced acceptordetails
2-Hydroxyglutarate + FAD → Oxoglutaric acid + FADHdetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Gene Name:
ADHFE1
Uniprot ID:
Q8IWW8
Molecular weight:
50307.42
Reactions
(S)-3-Hydroxybutyric acid + Oxoglutaric acid → Acetoacetic acid + D-2-Hydroxyglutaric aciddetails
4-Hydroxybutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + D-2-Hydroxyglutaric aciddetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
Not Available
Gene Name:
OGDHL
Uniprot ID:
Q9ULD0
Molecular weight:
Not Available
Reactions
Oxoglutaric acid + Thiamine pyrophosphate → 3-Carboxy-1-hydroxypropylthiamine diphosphate + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Important for recycling the chief excitatory neurotransmitter, glutamate, during neurotransmission.
Gene Name:
GLUD2
Uniprot ID:
P49448
Molecular weight:
61433.465
Reactions
L-Glutamic acid + Water + NAD(P)(+) → Oxoglutaric acid + Ammonia + NAD(P)Hdetails
L-Glutamic acid + NAD + Water → Oxoglutaric acid + Ammonia + NADH + Hydrogen Iondetails
L-Glutamic acid + NADP + Water → Oxoglutaric acid + Ammonia + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
AASS
Uniprot ID:
A4D0W4
Molecular weight:
102130.9
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA3
Uniprot ID:
Q7Z4N8
Molecular weight:
61125.675
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)details
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA1
Uniprot ID:
P13674
Molecular weight:
60966.645
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)details
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:
P4HA2
Uniprot ID:
O15460
Molecular weight:
60632.19
Reactions
L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)details
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD1
Uniprot ID:
Q02809
Molecular weight:
83549.55
Reactions
L-lysine-[procollagen] + Oxoglutaric acid + Oxygen → (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2)details
Protein lysine + Oxoglutaric acid + Oxygen → Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Waterdetails
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD2
Uniprot ID:
O00469
Molecular weight:
84685.07
Reactions
L-lysine-[procollagen] + Oxoglutaric acid + Oxygen → (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2)details
Protein lysine + Oxoglutaric acid + Oxygen → Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Waterdetails
General function:
Involved in oxidation reduction
Specific function:
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Gene Name:
TMLHE
Uniprot ID:
Q9NVH6
Molecular weight:
44048.895
Reactions
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-hydroxy-N(6),N(6),N(6)-trimethyl-L-lysine + Succinic acid + CO(2)details
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxidedetails
General function:
Involved in peptide-aspartate beta-dioxygenase activity
Specific function:
Isoform 1: specifically hydroxylates an Asp or Asn residue in certain epidermal growth factor-like (EGF) domains of a number of proteins. Isoform 8: membrane-bound Ca(2+)-sensing protein, which is a structural component of the ER-plasma membrane junctions. Isoform 8 regulates the activity of Ca(+2) released-activated Ca(+2) (CRAC) channels in T-cells.
Gene Name:
ASPH
Uniprot ID:
Q12797
Molecular weight:
83267.47
Reactions
Peptide L-aspartate + Oxoglutaric acid + Oxygen → peptide 3-hydroxy-L-aspartate + Succinic acid + CO(2)details
General function:
Involved in metal ion binding
Specific function:
Hydroxylates HIF-1 alpha at 'Asp-803' in the C-terminal transactivation domain (CAD). Functions as an oxygen sensor and, under normoxic conditions, the hydroxylation prevents interaction of HIF-1 with transcriptional coactivators including Cbp/p300-interacting transactivator. Involved in transcriptional repression through interaction with HIF1A, VHL and histone deacetylases. Hydroxylates specific Asn residues within ankyrin repeat domains (ARD) of NFKB1, NFKBIA, NOTCH1, ASB4, PPP1R12A and several other ARD-containing proteins. Also hydroxylates Asp and His residues within ARDs of ANK1 and TNKS2, respectively. Negatively regulates NOTCH1 activity, accelerating myogenic differentiation. Positively regulates ASB4 activity, promoting vascular differentiation.
Gene Name:
HIF1AN
Uniprot ID:
Q9NWT6
Molecular weight:
40285.25
Reactions
Hypoxia-inducible factor-L-asparagine + Oxoglutaric acid + Oxygen → hypoxia-inducible factor-(3S)-3-hydroxy-L-asparagine + Succinic acid + CO(2)details
Ankyrin-repeat-L-histidine + Oxoglutaric acid + Oxygen → ankyrin-repeat-(3S)-3-hydroxy-L-histidine + Succinic acid + CO(2)details
General function:
Involved in transferase activity, transferring nitrogenous groups
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
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
L-Kynurenine + Oxoglutaric acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
L-Cysteine + Oxoglutaric acid → 3-Mercaptopyruvic acid + DL-Glutamatedetails
Cysteic acid + Oxoglutaric acid → 3-Sulfopyruvic acid + L-Glutamic aciddetails
3-Sulfinoalanine + Oxoglutaric acid → B-Sulfinyl pyruvate + L-Glutamic aciddetails
4-Hydroxy-L-glutamic acid + Oxoglutaric acid → D-4-Hydroxy-2-oxoglutarate + L-Glutamic aciddetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Catalyzes the reversible transamination between alanine and 2-oxoglutarate to form pyruvate and glutamate.
Gene Name:
GPT2
Uniprot ID:
Q8TD30
Molecular weight:
46982.6
Reactions
L-Alanine + Oxoglutaric acid → Pyruvic acid + L-Glutamic aciddetails
General function:
Transcription
Specific function:
Not Available
Gene Name:
AADAT
Uniprot ID:
Q4W5N8
Molecular weight:
47351.2
General function:
Involved in transaminase activity
Specific function:
Not Available
Gene Name:
OAT
Uniprot ID:
P04181
Molecular weight:
48534.39
Reactions
Ornithine + Oxoglutaric acid → L-Glutamic gamma-semialdehyde + L-Glutamic aciddetails
General function:
Involved in 4-aminobutyrate transaminase activity
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
Reactions
Gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + L-Glutamic aciddetails
(S)-b-aminoisobutyric acid + Oxoglutaric acid → 2-Methyl-3-oxopropanoic acid + L-Glutamic aciddetails
Beta-Alanine + Oxoglutaric acid → Malonic semialdehyde + L-Glutamic aciddetails
(S)-b-aminoisobutyric acid + Oxoglutaric acid → (S)-Methylmalonic acid semialdehyde + L-Glutamic aciddetails
General function:
Involved in transaminase activity
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
Reactions
Glycine + Oxoglutaric acid → Glyoxylic acid + L-Glutamic aciddetails
General function:
Involved in catalytic 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:
38644.77
Reactions
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
General function:
Involved in catalytic 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:
33776.315
Reactions
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
L-Isoleucine + Oxoglutaric acid → 3-Methyl-2-oxovaleric acid + L-Glutamic aciddetails
L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
General function:
Involved in phosphoglycerate dehydrogenase activity
Specific function:
Not Available
Gene Name:
PHGDH
Uniprot ID:
O43175
Molecular weight:
56650.03
Reactions
2-Hydroxyglutarate + NAD → Oxoglutaric acid + NADHdetails
General function:
Involved in magnesium ion binding
Specific function:
Not Available
Gene Name:
IDH3B
Uniprot ID:
O43837
Molecular weight:
42183.39
Reactions
Isocitric acid + NAD → Oxoglutaric acid + CO(2) + NADHdetails
Isocitric acid + NAD → Oxoglutaric acid + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:
PLOD3
Uniprot ID:
O60568
Molecular weight:
84784.505
Reactions
L-lysine-[procollagen] + Oxoglutaric acid + Oxygen → (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2)details
Protein lysine + Oxoglutaric acid + Oxygen → Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Waterdetails
General function:
Involved in iron ion binding
Specific function:
Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Gene Name:
BBOX1
Uniprot ID:
O75936
Molecular weight:
44714.6
Reactions
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + CO(2)details
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxidedetails
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
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
Reactions
L-Tyrosine + Oxoglutaric acid → 4-Hydroxyphenylpyruvic acid + L-Glutamic aciddetails
L-Phenylalanine + Oxoglutaric acid → Phenylpyruvic acid + L-Glutamic aciddetails
2-Oxo-4-methylthiobutanoic acid + L-Glutamic acid → L-Methionine + Oxoglutaric aciddetails
General function:
Involved in 1-aminocyclopropane-1-carboxylate synthase activity
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
Reactions
L-Alanine + Oxoglutaric acid → Pyruvic acid + L-Glutamic aciddetails
General function:
Involved in magnesium ion binding
Specific function:
Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex.
Gene Name:
IDH2
Uniprot ID:
P48735
Molecular weight:
50908.915
Reactions
Isocitric acid + NADP → Oxoglutaric acid + CO(2) + NADPHdetails
Isocitric acid + NADP → Oxoglutaric acid + Carbon dioxide + NADPH + Hydrogen Iondetails
Oxalosuccinic acid → Oxoglutaric acid + Carbon dioxidedetails
General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:
DLD
Uniprot ID:
P09622
Molecular weight:
54176.91
General function:
Involved in metabolic process
Specific function:
Catalyzes the reversible conversion of 3-phosphohydroxypyruvate to phosphoserine and of 3-hydroxy-2-oxo-4-phosphonooxybutanoate to phosphohydroxythreonine (By similarity).
Gene Name:
PSAT1
Uniprot ID:
Q9Y617
Molecular weight:
35188.305
Reactions
Phosphoserine + Oxoglutaric acid → Phosphohydroxypyruvic acid + L-Glutamic aciddetails
O-Phospho-4-hydroxy-L-threonine + Oxoglutaric acid → 2-Oxo-3-hydroxy-4-phosphobutanoic acid + L-Glutamic aciddetails
General function:
Involved in electron carrier activity
Specific function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O14832
Molecular weight:
38538.065
Reactions
Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2)details
General function:
Involved in transferase activity, transferring nitrogenous groups
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 glyoxylate as amino-group acceptor (in vitro).
Gene Name:
AADAT
Uniprot ID:
Q8N5Z0
Molecular weight:
47351.17
Reactions
L-Kynurenine + Oxoglutaric acid → 4-(2-Aminophenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
Aminoadipic acid + Oxoglutaric acid → Oxoadipic acid + L-Glutamic aciddetails
L-3-Hydroxykynurenine + Oxoglutaric acid → 4-(2-Amino-3-hydroxyphenyl)-2,4-dioxobutanoic acid + L-Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.
Gene Name:
AASS
Uniprot ID:
Q9UDR5
Molecular weight:
102130.895
Reactions
N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP + Water → L-Lysine + Oxoglutaric acid + NADPHdetails
Saccharopine + NADP + Water → L-Lysine + Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in transferase activity, transferring nitrogenous groups
Specific function:
Not Available
Gene Name:
GOT1L1
Uniprot ID:
Q8NHS2
Molecular weight:
47304.72
Reactions
L-Aspartic acid + Oxoglutaric acid → Oxalacetic acid + L-Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation.
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Molecular weight:
57248.96
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Molecular weight:
54847.36
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P05091
Molecular weight:
56380.93
Reactions
2,5-Dioxopentanoate + NADP + Water → Oxoglutaric acid + NADPH + Hydrogen Iondetails
General function:
Not Available
Specific function:
Dioxygenase that repairs alkylated single-stranded DNA and RNA containing 3-methylcytosine by oxidative demethylation. Requires molecular oxygen, alpha-ketoglutarate and iron. May have a role in placental trophoblast lineage differentiation (By similarity). Has DNA lyase activity and introduces double-stranded breaks at abasic sites. Cleaves both single-stranded DNA and double-stranded DNA at abasic sites, with the greatest activity towards double-stranded DNA with two abasic sites. DNA lyase activity does not require alpha-ketoglutarate and iron.
Gene Name:
ALKBH1
Uniprot ID:
Q13686
Molecular weight:
43831.39
Reactions
DNA-base-CH(3) + Oxoglutaric acid + Oxygen → DNA-base + Formaldehyde + Succinic acid + CO(2)details
General function:
Involved in oxidoreductase activity
Specific function:
Dioxygenase that repairs alkylated DNA and RNA containing 1-methyladenine and 3-methylcytosine by oxidative demethylation. Can also repair alkylated DNA containing 1-ethenoadenine (in vitro). Has strong preference for double-stranded DNA. Has low efficiency with single-stranded substrates. Requires molecular oxygen, alpha-ketoglutarate and iron.
Gene Name:
ALKBH2
Uniprot ID:
Q6NS38
Molecular weight:
29322.22
Reactions
DNA-base-CH(3) + Oxoglutaric acid + Oxygen → DNA-base + Formaldehyde + Succinic acid + CO(2)details
General function:
Involved in catalytic activity
Specific function:
Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate.
Gene Name:
D2HGDH
Uniprot ID:
Q8N465
Molecular weight:
56415.58
Reactions
D-2-Hydroxyglutaric acid + acceptor → Oxoglutaric acid + reduced acceptordetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity).
Gene Name:
DHTKD1
Uniprot ID:
Q96HY7
Molecular weight:
103042.15
Reactions
Oxoglutaric acid + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine → [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)details
General function:
Involved in oxidoreductase activity
Specific function:
Cellular oxygen sensor that catalyzes, under normoxic conditions, the post-translational formation of 4-hydroxyproline in hypoxia-inducible factor (HIF) alpha proteins. Hydroxylates a specific proline found in each of the oxygen-dependent degradation (ODD) domains (N-terminal, NODD, and C-terminal, CODD) of HIF1A. Also hydroxylates HIF2A. Has a preference for the CODD site for both HIF1A and HIF1B. Hydroxylated HIFs are then targeted for proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Under hypoxic conditions, the hydroxylation reaction is attenuated allowing HIFs to escape degradation resulting in their translocation to the nucleus, heterodimerization with HIF1B, and increased expression of hypoxy-inducible genes. EGLN1 is the most important isozyme under normoxia and, through regulating the stability of HIF1, involved in various hypoxia-influenced processes such as angiogenesis in retinal and cardiac functionality.
Gene Name:
EGLN1
Uniprot ID:
Q9GZT9
Molecular weight:
46020.585
Reactions
Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen → hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2)details
General function:
Involved in oxidoreductase activity
Specific function:
Cellular oxygen sensor that catalyzes, under normoxic conditions, the post-translational formation of 4-hydroxyproline in hypoxia-inducible factor (HIF) alpha proteins. Hydroxylates a specific proline found in each of the oxygen-dependent degradation (ODD) domains (N-terminal, NODD, and C-terminal, CODD) of HIF1A. Also hydroxylates HIF2A. Has a preference for the CODD site for both HIF1A and HIF2A. Hydroxylated HIFs are then targeted for proteasomal degradation via the von Hippel-Lindau ubiquitination complex. Under hypoxic conditions, the hydroxylation reaction is attenuated allowing HIFs to escape degradation resulting in their translocation to the nucleus, heterodimerization with HIF1B, and increased expression of hypoxy-inducible genes. EGLN2 is involved in regulating hypoxia tolerance and apoptosis in cardiac and skeletal muscle. Also regulates susceptibility to normoxic oxidative neuronal death.
Gene Name:
EGLN2
Uniprot ID:
Q96KS0
Molecular weight:
43650.03
Reactions
Hypoxia-inducible factor-L-proline + Oxoglutaric acid + Oxygen → hypoxia-inducible factor-trans-4-hydroxy-L-proline + Succinic acid + CO(2)details

Only showing the first 50 proteins. There are 68 proteins in total.