Reaction: (R)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2
- in pathway: Interconversion of 2-oxoglutarate and 2-hydroxyglutarate
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)).
Reaction - small molecule participants:
2OG [mitochondrial matrix]
FADH2 [mitochondrial matrix]
FAD [mitochondrial matrix]
2HG [mitochondrial matrix]
Reactome.org reaction link: R-HSA-880007
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Reaction input - small molecules:
FAD(3-)
(R)-2-hydroxyglutaric acid
Reaction output - small molecules:
2-oxoglutarate(2-)
FADH2(2-)
Reactome.org link: R-HSA-880007