Record Information
Version1.0
Creation date2020-04-20 19:57:12 UTC
Update date2020-09-17 15:40:37 UTC
Primary IDFDB112367
Secondary Accession NumbersNot Available
Chemical Information
FooDB Name2-Hydroxyglutarate
Description2-Hydroxyglutarate 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. 2-Hydroxyglutarate is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. 2-Hydroxyglutarate exists in all living species, ranging from bacteria to humans. 2-hydroxyglutarate can be biosynthesized from oxoglutaric acid through the action of the enzyme L-2-hydroxyglutarate dehydrogenase, mitochondrial. In humans, 2-hydroxyglutarate is involved in the metabolic disorder called the oncogenic action of d-2-hydroxyglutarate in hydroxyglutaric aciduria. In cancer it is either produced by overexpression of phosphoglycerate dehydrogenase (PHGDH) or is produced in excess by gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). A deficiency in either of these two enzymes can lead to a disease known as 2-hydroxyglutaric aciduria. 2-Hydroxyglutarate exists in 2 isomers: L-2-hydroxyglutarate acid and D-2-hydroxyglutarate. D-2-hydroxyglutaric aciduria (caused by loss of D2HGDH or gain of function of IDH) is rare, with symptoms including cancer, macrocephaly, cardiomyopathy, mental retardation, hypotonia, and cortical blindness. 2-Hydroxyglutarate is a potentially toxic compound. 2-Hydroxyglutarate, with regard to humans, has been found to be associated with several diseases such as deafness, onychodystrophy, osteodystrophy, mental retardation, and seizures syndrome, d, l-2-hydroxyglutaric aciduria, supragingival plaque, and colorectal cancer; 2-hydroxyglutarate has also been linked to the inborn metabolic disorder l-2-hydroxyglutaric aciduria. 2-Hydroxyglutarate has also been found to be a metabolite in Aspergillus (PMID: 6057807). 2-hydroxyglutarate is also the product of gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). In humans it is part of butanoate metabolic pathway and can be produced by phosphoglycerate dehydrogenase (PHGDH). Humans have to variants of this enzyme: 2-Hydroxyglutarate dehydrogenase (D2HGDH) and L-2-hydroxyglutarate dehydrogenase (L2HGDH).
CAS NumberNot Available
Structure
Thumb
Synonyms
SynonymSource
alpha-Hydroxyglutaric acidChEBI
alpha-HydroxyglutarateKegg
a-HydroxyglutarateGenerator
a-Hydroxyglutaric acidGenerator
Α-hydroxyglutarateGenerator
Α-hydroxyglutaric acidGenerator
2-Hydroxyglutaric acidGenerator
D-2-HydroxyglutarateHMDB
alpha-Hydroxyglutarate, (D)-isomerHMDB
alpha-Hydroxyglutarate, (DL)-isomerHMDB
alpha-Hydroxyglutarate, (L)-isomerHMDB
alpha-Hydroxyglutarate, disodium saltHMDB
(±)-2-hydroxyglutaric acidHMDB
2,3-Dideoxypentaric acidHMDB
2-Hydroxypentanedioic acidHMDB
DL-2-Hydroxyglutaric acidHMDB
2-HydroxyglutarateGenerator
Predicted PropertiesNot Available
Chemical FormulaC5H8O5
IUPAC name
InChI IdentifierInChI=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
Isomeric SMILESOC(CCC(O)=O)C(O)=O
Average Molecular Weight148.114
Monoisotopic Molecular Weight148.037173366
Classification
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
OntologyNo ontology term
Physico-Chemical Properties - Experimental
Physico-Chemical Properties - Experimental
PropertyValueReference
Physical stateNot Available
Physical DescriptionNot Available
Mass CompositionNot Available
Melting PointNot Available
Boiling PointNot Available
Experimental Water SolubilityNot Available
Experimental logPNot Available
Experimental pKaNot Available
Isoelectric pointNot Available
ChargeNot Available
Optical RotationNot Available
Spectroscopic UV DataNot Available
DensityNot Available
Refractive IndexNot Available
Spectra
Spectra
EI-MS/GC-MSNot Available
MS/MSNot Available
NMRNot Available
ChemSpider IDNot Available
ChEMBL IDNot Available
KEGG Compound IDNot Available
Pubchem Compound IDNot Available
Pubchem Substance IDNot Available
ChEBI IDNot Available
Phenol-Explorer IDNot Available
DrugBank IDNot Available
HMDB IDNot Available
CRC / DFC (Dictionary of Food Compounds) IDNot Available
EAFUS IDNot Available
Dr. Duke IDNot Available
BIGG IDNot Available
KNApSAcK IDNot Available
HET IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
Flavornet IDNot Available
GoodScent IDNot Available
SuperScent IDNot Available
Wikipedia IDNot Available
Phenol-Explorer Metabolite IDNot Available
Duplicate IDSNot Available
Old DFC IDSNot Available
Associated Foods
FoodContent Range AverageReference
FoodReference
Biological Effects and Interactions
Health Effects / BioactivitiesNot Available
EnzymesNot Available
PathwaysNot Available
MetabolismNot Available
BiosynthesisNot Available
Organoleptic Properties
FlavoursNot Available
Files
MSDSNot Available
References
Synthesis ReferenceNot Available
General ReferenceNot Available
Content Reference