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

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

Stable Identifier

R-HSA-880007

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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

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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
15070399	Identification of a dehydrogenase acting on D-2-hydroxyglutarate Rider, MH, Achouri, Y, Vertommen, D, Noel, G, Veiga-da-Cunha, M, Van Schaftingen, E	Biochem J	2004
7564244	D-2-hydroxyglutaric acidaemia: identification of a new enzyme, D-2-hydroxyglutarate dehydrogenase, localized in mitochondria Wanders, RJA, Mooyer, P	J Inherit Metab Dis	1995
16435184	Kinetic characterization of human hydroxyacid-oxoacid transhydrogenase: relevance to D-2-hydroxyglutaric and gamma-hydroxybutyric acidurias Verhoeven, NM, Wickenhagen, WV, Ten Brink, HJ, Gibson, KM, Struys, EA, Jakobs, C	J Inherit Metab Dis	2005