In enzymology , a D-2-hydroxyglutarate dehydrogenase (EC 1.1.99.39 ) is an enzyme that catalyzes the chemical reaction
(R)-2-hydroxyglutarate + acceptor
⇌ ⇌ -->
{\displaystyle \rightleftharpoons }
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
^ 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 1133759 . PMID 15070399 .
^ 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 2757207 . PMID 19586914 .
^ 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 2899879 . PMID 20501910 .
^ 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 3064194 . PMID 21296880 .
Further reading
Activity Regulation Classification Kinetics Types