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Showing metabocard for 2-Hydroxyglutarate (HMDB0059655)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2012-10-30 10:32:48 UTC
Update Date2023-05-30 20:55:56 UTC
HMDB IDHMDB0059655
Secondary Accession Numbers
  • HMDB59655
Metabolite Identification
Common Name2-Hydroxyglutarate
Description
Structure
Thumb
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0059655 (2-Hydroxyglutarate)

  Mrv1652309042000402D          

 10  9  0  0  0  0            999 V2000
 9999.2646 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9999.979310000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.6939 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10002.1250 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8349 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.2646 9998.9638    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  0  0  0  0
  1 10  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  7  8  1  0  0  0  0
  7  9  2  0  0  0  0
M  END
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3D MOL for HMDB0059655 (2-Hydroxyglutarate)

HMDB0059655
     RDKit          3D
2-Hydroxyglutarate
 18 17  0  0  0  0  0  0  0  0999 V2000
    3.0317   -1.0607    0.2792 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3638   -0.0196    0.1451 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8559    1.1002   -0.5141 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9655    0.0897    0.6959 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1013   -0.6685   -0.2877 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3473   -0.6983    0.0686 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9678   -1.4456   -0.9647 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9167    0.6701    0.0149 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7653    0.9961   -0.8852 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5910    1.6824    0.9064 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5718    1.0602   -1.2099 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9122   -0.3792    1.7028 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6495    1.1467    0.7231 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1879   -0.0894   -1.2531 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5364   -1.6704   -0.4715 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5183   -1.1992    1.0381 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5178   -2.1661   -0.5702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5517    2.6517    0.5823 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  6  8  1  0
  8  9  2  0
  8 10  1  0
  3 11  1  0
  4 12  1  0
  4 13  1  0
  5 14  1  0
  5 15  1  0
  6 16  1  0
  7 17  1  0
 10 18  1  0
M  END
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3D SDF for HMDB0059655 (2-Hydroxyglutarate)

  Mrv1652309042000402D          

 10  9  0  0  0  0            999 V2000
 9999.2646 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9999.979310000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.6939 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10002.1250 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8349 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.2646 9998.9638    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  0  0  0  0
  1 10  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  7  8  1  0  0  0  0
  7  9  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0059655

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(CCC(O)=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)

> <INCHI_KEY>
HWXBTNAVRSUOJR-UHFFFAOYSA-N

> <FORMULA>
C5H8O5

> <MOLECULAR_WEIGHT>
148.114

> <EXACT_MASS>
148.037173366

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
18

> <JCHEM_AVERAGE_POLARIZABILITY>
12.97429694878232

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
3

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-hydroxypentanedioic acid

> <ALOGPS_LOGP>
-1.04

> <JCHEM_LOGP>
-0.824980223

> <ALOGPS_LOGS>
0.03

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
4.159324461588801

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.276485914460605

> <JCHEM_PKA_STRONGEST_BASIC>
-3.8068384384876675

> <JCHEM_POLAR_SURFACE_AREA>
94.83000000000001

> <JCHEM_REFRACTIVITY>
29.6302

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.57e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
2-hydroxyglutaric acid

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0059655 (2-Hydroxyglutarate)

HMDB0059655
     RDKit          3D
2-Hydroxyglutarate
 18 17  0  0  0  0  0  0  0  0999 V2000
    3.0317   -1.0607    0.2792 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3638   -0.0196    0.1451 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8559    1.1002   -0.5141 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9655    0.0897    0.6959 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1013   -0.6685   -0.2877 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3473   -0.6983    0.0686 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9678   -1.4456   -0.9647 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9167    0.6701    0.0149 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7653    0.9961   -0.8852 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5910    1.6824    0.9064 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5718    1.0602   -1.2099 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9122   -0.3792    1.7028 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6495    1.1467    0.7231 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1879   -0.0894   -1.2531 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5364   -1.6704   -0.4715 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5183   -1.1992    1.0381 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5178   -2.1661   -0.5702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5517    2.6517    0.5823 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  6  8  1  0
  8  9  2  0
  8 10  1  0
  3 11  1  0
  4 12  1  0
  4 13  1  0
  5 14  1  0
  5 15  1  0
  6 16  1  0
  7 17  1  0
 10 18  1  0
M  END
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PDB for HMDB0059655 (2-Hydroxyglutarate)

HEADER    PROTEIN                                 04-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   04-SEP-20         0                                  
HETATM    1  C   UNK     0    8665.2948666.273   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8666.6288667.042   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8667.9628666.273   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8669.3018667.042   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8670.6338666.273   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8669.3018668.582   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0    8663.9608667.042   0.000  0.00  0.00           C+0
HETATM    8  O   UNK     0    8662.6258666.273   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0    8663.9608668.582   0.000  0.00  0.00           O+0
HETATM   10  O   UNK     0    8665.2948664.732   0.000  0.00  0.00           O+0
CONECT    1    2    7   10                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4                                                       
CONECT    4    3    5    6                                                  
CONECT    5    4                                                            
CONECT    6    4                                                            
CONECT    7    1    8    9                                                  
CONECT    8    7                                                            
CONECT    9    7                                                            
CONECT   10    1                                                            
MASTER        0    0    0    0    0    0    0    0   10    0   18    0
END
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3D PDB for HMDB0059655 (2-Hydroxyglutarate)

COMPND    HMDB0059655
HETATM    1  O1  UNL     1       3.032  -1.061   0.279  1.00  0.00           O  
HETATM    2  C1  UNL     1       2.364  -0.020   0.145  1.00  0.00           C  
HETATM    3  O2  UNL     1       2.856   1.100  -0.514  1.00  0.00           O  
HETATM    4  C2  UNL     1       0.966   0.090   0.696  1.00  0.00           C  
HETATM    5  C3  UNL     1       0.101  -0.669  -0.288  1.00  0.00           C  
HETATM    6  C4  UNL     1      -1.347  -0.698   0.069  1.00  0.00           C  
HETATM    7  O3  UNL     1      -1.968  -1.446  -0.965  1.00  0.00           O  
HETATM    8  C5  UNL     1      -1.917   0.670   0.015  1.00  0.00           C  
HETATM    9  O4  UNL     1      -2.765   0.996  -0.885  1.00  0.00           O  
HETATM   10  O5  UNL     1      -1.591   1.682   0.906  1.00  0.00           O  
HETATM   11  H1  UNL     1       3.572   1.060  -1.210  1.00  0.00           H  
HETATM   12  H2  UNL     1       0.912  -0.379   1.703  1.00  0.00           H  
HETATM   13  H3  UNL     1       0.650   1.147   0.723  1.00  0.00           H  
HETATM   14  H4  UNL     1       0.188  -0.089  -1.253  1.00  0.00           H  
HETATM   15  H5  UNL     1       0.536  -1.670  -0.472  1.00  0.00           H  
HETATM   16  H6  UNL     1      -1.518  -1.199   1.038  1.00  0.00           H  
HETATM   17  H7  UNL     1      -2.518  -2.166  -0.570  1.00  0.00           H  
HETATM   18  H8  UNL     1      -1.552   2.652   0.582  1.00  0.00           H  
CONECT    1    2    2
CONECT    2    3    4
CONECT    3   11
CONECT    4    5   12   13
CONECT    5    6   14   15
CONECT    6    7    8   16
CONECT    7   17
CONECT    8    9    9   10
CONECT   10   18
END
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SMILES for HMDB0059655 (2-Hydroxyglutarate)

OC(CCC(O)=O)C(O)=O
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INCHI for HMDB0059655 (2-Hydroxyglutarate)

InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC5H8O5
Average Molecular Weight148.114
Monoisotopic Molecular Weight148.037173366
IUPAC Name2-hydroxypentanedioic acid
Traditional Name2-hydroxyglutaric acid
CAS Registry Number2889-31-8
SMILES
OC(CCC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)
InChI KeyHWXBTNAVRSUOJR-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain hydroxy acids and derivatives. These are hydroxy acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassShort-chain hydroxy acids and derivatives
Direct ParentShort-chain hydroxy acids and derivatives
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Fatty acid
  • Monosaccharide
  • Dicarboxylic acid or derivatives
  • Alpha-hydroxy acid
  • Secondary alcohol
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Industrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Brain
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
D-2-hydroxyglutaric aciduria
  1. Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed:8134166 ]
  2. Wajne M, Vargas CR, Funayama C, Fernandez A, Elias ML, Goodman SI, Jakobs C, van der Knaap MS: D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings. J Inherit Metab Dis. 2002 Feb;25(1):28-34. [PubMed:11999977 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
L-2-Hydroxyglutaric aciduria
  1. Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed:8134166 ]
  2. Barbot C, Fineza I, Diogo L, Maia M, Melo J, Guimaraes A, Pires MM, Cardoso ML, Vilarinho L: L-2-Hydroxyglutaric aciduria: clinical, biochemical and magnetic resonance imaging in six Portuguese pediatric patients. Brain Dev. 1997 Jun;19(4):268-73. [PubMed:9187477 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Supragingival Plaque
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Glutaric aciduria II
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
D, L-2-hydroxyglutaric aciduria
  1. Muntau AC, Roschinger W, Merkenschlager A, van der Knaap MS, Jakobs C, Duran M, Hoffmann GF, Roscher AA: Combined D-2- and L-2-hydroxyglutaric aciduria with neonatal onset encephalopathy: a third biochemical variant of 2-hydroxyglutaric aciduria? Neuropediatrics. 2000 Jun;31(3):137-40. doi: 10.1055/s-2000-7497. [PubMed:10963100 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Cerebrotendinous xanthomatosis
  1. Siman-Tov T, Meiner V, Gadoth N: Could steroids mask the diagnosis of cerebrotendinous xanthomatosis? J Neurol Sci. 2006 Apr 15;243(1-2):83-6. Epub 2006 Jan 30. [PubMed:16445943 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Vilarinho L, Marques JS, Rocha H, Ramos A, Lopes L, Narayan SB, Bennett MJ: Diagnosis of a patient with a kinetic variant of medium and short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency by newborn screening. Mol Genet Metab. 2012 Jul;106(3):277-80. doi: 10.1016/j.ymgme.2012.04.005. Epub 2012 Apr 13. [PubMed:22579592 ]
Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome
  1. James AW, Miranda SG, Culver K, Hall BD, Golabi M: DOOR syndrome: clinical report, literature review and discussion of natural history. Am J Med Genet A. 2007 Dec 1;143A(23):2821-31. doi: 10.1002/ajmg.a.32054. [PubMed:17994565 ]
Associated OMIM IDs
  • 600721 (D-2-hydroxyglutaric aciduria)
  • 236792 (L-2-Hydroxyglutaric aciduria)
  • 114500 (Colorectal cancer)
  • 231680 (Glutaric aciduria II)
  • 610247 (Eosinophilic esophagitis)
  • 615182 (D, L-2-hydroxyglutaric aciduria)
  • 213700 (Cerebrotendinous xanthomatosis)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 220500 (Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDNot Available
KNApSAcK IDNot Available
Chemspider ID42
KEGG Compound IDC02630
BioCyc ID2-HYDROXYGLUTARIC_ACID
BiGG IDNot Available
Wikipedia LinkAlpha-Hydroxyglutaric_acid
METLIN IDNot Available
PubChem Compound43
PDB IDNot Available
ChEBI ID17084
Food Biomarker OntologyNot Available
VMH IDM00653
MarkerDB IDMDB00029834
Good Scents IDNot Available
References
Synthesis ReferenceKobayashi, Hidehiko; Yamaguchi, Koretaka; Yamashita, Takeshi. a-Hydroxyglutaric acid from glutamic acid. Jpn. Tokkyo Koho (1968), 3 pp.
Material Safety Data Sheet (MSDS)Not Available
General References

Enzymes

Enzyme Details
1. D-3-phosphoglycerate dehydrogenase
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
Enzyme Details
2. D-2-hydroxyglutarate dehydrogenase, mitochondrial
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
Enzyme Details
3. L-2-hydroxyglutarate dehydrogenase, mitochondrial
General function:
Involved in 2-hydroxyglutarate dehydrogenase activity
Specific function:
Not Available
Gene Name:
L2HGDH
Uniprot ID:
Q9H9P8
Molecular weight:
50315.58
Reactions
2-Hydroxyglutarate + FAD → Oxoglutaric acid + FADHdetails
Enzyme Details
4. Hydroxyacid-oxoacid transhydrogenase, mitochondrial
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