Reaction: (S)-2-hydroxyglutarate + FAD => 2-oxoglutarate + FADH2
- in pathway: Interconversion of 2-oxoglutarate and 2-hydroxyglutarate
L2HDGH associated with the mitochondrial inner membrane catalyzes the FAD-dependent reaction of (S)-2-hydroxyglutarate to form 2-oxoglutarate (Rzem et al. 2006). L2HDGH mutations are associated with high levels of (S)-2-hydroxyglutarate in vivo and variable neurological symptoms (Rzem et al. 2004; Topcu et al. 2004).
Reaction - small molecule participants:
2OG [mitochondrial matrix]
FADH2 [mitochondrial matrix]
FAD [mitochondrial matrix]
2HG [mitochondrial matrix]
Reactome.org reaction link: R-HSA-880050
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Reaction input - small molecules:
FAD(3-)
(S)-2-hydroxyglutaric acid
Reaction output - small molecules:
2-oxoglutarate(2-)
FADH2(2-)
Reactome.org link: R-HSA-880050