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MT001545

UniProt Annotations

Entry Information

Gene Name	alcohol dehydrogenase, iron containing, 1
Protein Entry	HOT_HUMAN
UniProt ID	Q8IWW8
Species	Human

Comments

Comment type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=4; Name=1; IsoId=Q8IWW8-1; Sequence=Displayed; Name=2; IsoId=Q8IWW8-2; Sequence=VSP_031984; Name=3; IsoId=Q8IWW8-3; Sequence=VSP_031985, VSP_031986; Note=No experimental confirmation available.; Name=4; IsoId=Q8IWW8-4; Sequence=VSP_031984, VSP_031985, VSP_031986; Note=No experimental confirmation available.;
Biophysicochemical Properties	Kinetic parameters: KM=0.17 mM for GHB {ECO:0000269\|PubMed:16435184}; KM=1.2 mM for 2-KG {ECO:0000269\|PubMed:16435184}; KM=0.12 mM for D-2-HG {ECO:0000269\|PubMed:16435184}; KM=0.04 mM for SSA {ECO:0000269\|PubMed:16435184}; KM=0.8 mM for L-3-OHB {ECO:0000269\|PubMed:16435184}; Vmax=16 nmol/h/mg enzyme with GHB and 2-KG as substrates {ECO:0000269\|PubMed:16435184}; Vmax=0.5 nmol/h/mg enzyme with SSA and D-2-HG as substrates {ECO:0000269\|PubMed:16435184}; Vmax=1.8 nmol/h/mg enzyme with L-3-OHB and 2-KG as substrates {ECO:0000269\|PubMed:16435184}; pH dependence: Optimum pH is 7.5. {ECO:0000269\|PubMed:16435184};
Catalytic Activity	4-hydroxybutanoate + 2-oxoglutarate = succinic semialdehyde + (R)-2-hydroxyglutarate. {ECO:0000269\|PubMed:16435184}.
Catalytic Activity	(S)-3-hydroxybutanoate + 2-oxoglutarate = acetoacetate + (R)-2-hydroxyglutarate. {ECO:0000269\|PubMed:16435184}.
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. {ECO:0000269\|PubMed:16435184}.
Similarity	Belongs to the iron-containing alcohol dehydrogenase family. Hydroxyacid-oxoacid transhydrogenase subfamily. {ECO:0000305}.
Subcellular Location	Mitochondrion {ECO:0000250}.
Tissue Specificity	Only expressed in adult liver. {ECO:0000269\|PubMed:12592711}.