Reaction: (R)-2-hydroxyglutarate + succinate semialdehyde <=> 2-oxoglutarate + 4-hydroxybutyrate
- in pathway: Interconversion of 2-oxoglutarate and 2-hydroxyglutarate
ADHFE1 catalyzes the reversible reaction of (R)-2-hydroxyglutarate and succinate semialdehyde to form 2-oxoglutarate and 4-hydroxybutyrate (Struys et al. 2005). The localization of human ADHFE1 to the mitochondrial matrix is inferred from the location determined experimentally for its rat homolog (Kaufman et al. 1988).
Reaction - small molecule participants:
2OG [mitochondrial matrix]
HBUT [mitochondrial matrix]
SUCCSA [mitochondrial matrix]
2HG [mitochondrial matrix]
Reactome.org reaction link: R-HSA-880002
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Reaction input - small molecules:
4-oxobutanoate
(R)-2-hydroxyglutaric acid
Reaction output - small molecules:
2-oxoglutarate(2-)
4-hydroxybutyrate
Reactome.org link: R-HSA-880002