HOT

Hydroxyacid-oxoacid transhydrogenase, mitochondrial; Isoform 2 of Hydroxyacid-oxoacid transhydrogenase, mitochondrial

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.

    Matrix Type

    • Tissue/Cells

    Gene Symbol

    • ADHFE1

    UniProt ID

    • Q8IWW8

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