hydroxyacid-oxoacid transhydrogenase, mitochondrial (HOT, Fe-containing alcohol dehydrogenase, ADHFe1, alcohol dehydrogenase iron-containing protein 1, ADHFE1)

Function: - catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate to succinic semialdehyde coupled to reduction of 2-ketoglutarate to D-2-hydroxyglutarate - D,L-3-hydroxyisobutyrate & L-3-hydroxybutyrate are also substrates for ADHFE1 with 10-fold lower activities (S)-3-hydroxybutanoate + 2-oxoglutarate acetoacetate + (R)-2-hydroxyglutarate 2-oxoglutaric acid + 4-hydroxybutanoic acid (R)-2-hydroxyglutaric acid + succinic semialdehyde Kinetic parameters: - KM=0.17 mM for gamma-hydroxybutyrate - KM=1.2 mM for 2-ketoglutarate - KM=0.12 mM for D-2-hydroxyglutarate - KM=0.04 mM for succinic semialdehyde - KM=0.8 mM for L-3-hydroxybutyrate - Vmax=16 nmol/h/mg enzyme with gamma-hydroxybutyrate & 2-ketoglutarate as substrates - Vmax=0.5 nmol/h/mg enzyme with succinic semialdehyde & D-2-hydroxyglutarate as substrates - Vmax=1.8 nmol/h/mg enzyme with L-3-hydroxybutyrate & 2-ketoglutarate as substrates - Optimum pH is 7.5 Structure: - belongs to the iron-containing alcohol dehydrogenase family, hydroxyacid-oxoacid transhydrogenase subfamily Compartment: mitochondria (putative) Alternative splicing: named isoforms=4 Expression: only expressed in adult liver

General

mitochondrial protein oxidoreductase

Properties

SIZE: entity length = 467 aa MW = 50 kD COMPARTMENT: mitochondria MOTIF: active site

Database Correlations

UniProt Q8IWW8 Pfam PF00465 Kegg hsa:1378 ENZYME 1.1.99.24

References

UniProt :accession Q8IWW8