D-2-hydroxyglutarate dehydrogenase

D-2-hydroxyglutarate dehydrogenase
Identifiers
EC number 1.1.99.39
Databases
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum

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

(R)-2-hydroxyglutarate + acceptor 2-oxoglutarate + reduced acceptor

Thus, the two substrates of this enzyme are (R)-2-hydroxyglutarate and acceptor, whereas its two products are 2-oxoglutarate and reduced acceptor.

The enzyme activity has been confirmed in animals[1] as well as in plants .[2]

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.[3][4]

See also

References

  1. ↑ Achouri Y, Noël G, Vertommen D, Rider MH, Veiga-Da-Cunha M, Van Schaftingen E (July 2004). "Identification of a dehydrogenase acting on D-2-hydroxyglutarate". Biochem. J. 381 (Pt 1): 35–42. doi:10.1042/BJ20031933. PMC 1133759Freely accessible. PMID 15070399.
  2. ↑ Engqvist M, Drincovich MF, Flügge UI, Maurino VG (September 2009). "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. doi:10.1074/jbc.M109.021253. PMC 2757207Freely accessible. PMID 19586914.
  3. ↑ Araújo WL, Ishizaki K, Nunes-Nesi A, Larson TR, Tohge T, Krahnert I, Witt S, Obata T, Schauer N, Graham IA, Leaver CJ, Fernie AR (May 2010). "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. doi:10.1105/tpc.110.075630. PMC 2899879Freely accessible. PMID 20501910.
  4. ↑ Engqvist MK, Kuhn A, Wienstroer J, Weber K, Jansen EE, Jakobs C, Weber AP, Maurino VG (April 2011). "Plant D-2-hydroxyglutarate dehydrogenase participates in the catabolism of lysine especially during senescence". J Biol Chem. 286 (April 1): 11382–11390. doi:10.1074/jbc.M110.194175. PMC 3064194Freely accessible. PMID 21296880.

Further reading


This article is issued from Wikipedia - version of the 5/25/2016. The text is available under the Creative Commons Attribution/Share Alike but additional terms may apply for the media files.