(R)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2

Stable Identifier
R-HSA-880007
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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D2HDGH associated with the mitochondrial inner membrane catalyzes the FAD-dependent reaction of (R)-2-hydroxyglutarate to form 2-oxoglutarate (Achouri et al. 2004). D2HDGH mutations are associated with high levels of (R)-2-hydroxyglutarate in vivo and variable neurological symptoms (Struys et al. 2005; Wanders and Mooyer 1995)).

Literature References
PubMed ID Title Journal Year
7564244 D-2-hydroxyglutaric acidaemia: identification of a new enzyme, D-2-hydroxyglutarate dehydrogenase, localized in mitochondria

Wanders, RJ, Mooyer, P

J Inherit Metab Dis 1995
15070399 Identification of a dehydrogenase acting on D-2-hydroxyglutarate

Achouri, Y, Noel, G, Vertommen, D, Rider, MH, Veiga-da-Cunha, M, Van Schaftingen, E

Biochem J 2004
16435184 Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias

Struys, EA, Verhoeven, NM, Ten Brink, HJ, Wickenhagen, WV, Gibson, KM, Jakobs, C

J Inherit Metab Dis 2005
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
(R)-2-hydroxyglutarate dehydrogenase activity of D2HGDH [mitochondrial matrix]
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Activity
This event is regulated
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