Protein Name:
Hydroxyacid-oxoacid transhydrogenase, mitochondrial
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UniprotKB/SwissProt ID: Q8IWW8 (Q8IWW8)
Gene Name:
ADHFE1
Organism: Homo sapiens (Human)
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
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion
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Graphical Visualization of S-nitrosylation Sites:
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