ADHFE1
Function
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Sequence Similarities
Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily.
Tissue Specificity
Only expressed in adult liver.
Cellular localization
- Mitochondrion
Alternative names
HMFT2263, ADHFE1, HOT, Alcohol dehydrogenase iron-containing protein 1, Fe-containing alcohol dehydrogenase, ADHFe1