From Wikipedia, the free encyclopedia
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