ID:HOT_HUMAN DESCRIPTION: RecName: Full=Hydroxyacid-oxoacid transhydrogenase, mitochondrial; Short=HOT; EC=1.1.99.24; AltName: Full=Alcohol dehydrogenase iron-containing protein 1; Short=ADHFe1; AltName: Full=Fe-containing alcohol dehydrogenase; Flags: Precursor; 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. CATALYTIC ACTIVITY: (S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate. CATALYTIC ACTIVITY: 4-hydroxybutanoate + 2-oxoglutarate = succinic semialdehyde + (R)-2-hydroxyglutarate. BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.17 mM for GHB; KM=1.2 mM for 2-KG; KM=0.12 mM for D-2-HG; KM=0.04 mM for SSA; KM=0.8 mM for L-3-OHB; Vmax=16 nmol/h/mg enzyme with GHB and 2-KG as substrates; Vmax=0.5 nmol/h/mg enzyme with SSA and D-2-HG as substrates; Vmax=1.8 nmol/h/mg enzyme with L-3-OHB and 2-KG as substrates; pH dependence: Optimum pH is 7.5; SUBCELLULAR LOCATION: Mitochondrion (By similarity). TISSUE SPECIFICITY: Only expressed in adult liver. SIMILARITY: Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q8IWW8
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Gene Ontology (GO) Annotations with Structured Vocabulary
Molecular Function: GO:0004022 alcohol dehydrogenase (NAD) activity GO:0016491 oxidoreductase activity GO:0046872 metal ion binding GO:0047988 hydroxyacid-oxoacid transhydrogenase activity
Biological Process: GO:0006103 2-oxoglutarate metabolic process GO:0006539 glutamate catabolic process via 2-oxoglutarate GO:0055114 oxidation-reduction process
US Server
