Biology:D-2-hydroxyglutarate dehydrogenase

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Short description: Class of enzymes
D-2-hydroxyglutarate dehydrogenase
Identifiers
EC number1.1.99.39
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum

In enzymology, D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39) is an enzyme that catalyzes the chemical reaction

  1. REDIRECT Template:Chemical reaction

The two substrates of this enzyme are (R)-2-hydroxyglutaric acid and an electron acceptor. Its products are α-ketoglutaric acid and the corresponding reduced acceptor.[1]

The enzyme activity has been confirmed in animals[2] as well as in plants.[3]

Nomenclature

This enzyme belongs to the family of oxidoreductases, specifically those acting on the CH-OH group of donor with other acceptors. The systematic name of this enzyme class is (R)-2-hydroxyglutarate:acceptor 2-oxidoreductase. Other names in common use include:

  • (R)-2-hydroxyglutarate:(acceptor) 2-oxidoreductase
  • alpha-hydroxyglutarate dehydrogenase
  • alpha-hydroxyglutarate dehydrogenase (NAD+ specific)
  • alpha-hydroxyglutarate oxidoreductase
  • alpha-ketoglutarate reductase
  • hydroxyglutaric dehydrogenase
  • D-alpha-hydroxyglutarate dehydrogenase
  • D-alpha-hydroxyglutarate:NAD+ 2-oxidoreductase

Clinical significance

Deficiency in this enzyme in humans (D2HGDH) or in the model plant Arabidopsis thaliana (At4g36400) leads to massive accumulation of D-2-hydroxyglutarate. In humans this results in the fatal neurometabolic disorder 2-hydroxyglutaric aciduria whereas plants seem to be to a large extent unaffected by high cellular concentrations of this compound.[4][5]

See also

References

  1. ↑ Enzyme 1.1.99.39 at KEGG Pathway Database.
  2. ↑ "Identification of a dehydrogenase acting on D-2-hydroxyglutarate". Biochem. J. 381 (Pt 1): 35–42. July 2004. doi:10.1042/BJ20031933. PMID 15070399. 
  3. ↑ "Two D-2-hydroxy-acid dehydrogenases in Arabidopsis thaliana with catalytic capacities to participate in the last reactions of the methylglyoxal and beta-oxidation pathways". J. Biol. Chem. 284 (37): 25026–37. September 2009. doi:10.1074/jbc.M109.021253. PMID 19586914. 
  4. ↑ "Identification of the 2-hydroxyglutarate and isovaleryl-CoA dehydrogenases as alternative electron donors linking lysine catabolism to the electron transport chain of Arabidopsis mitochondria". Plant Cell 22 (5): 1549–63. May 2010. doi:10.1105/tpc.110.075630. PMID 20501910. Bibcode: 2010PlanC..22.1549A. 
  5. ↑ "Plant D-2-hydroxyglutarate dehydrogenase participates in the catabolism of lysine especially during senescence". J Biol Chem 286 (April 1): 11382–11390. April 2011. doi:10.1074/jbc.M110.194175. PMID 21296880. 

Further reading




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