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Hydroxyacid-oxoacid transhydrogenase, mitochondrial

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Protein Overview: 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.

Synonyms: HOT, Alcohol dehydrogenase iron-containing protein 1, ADHFe1

Gene name: ADHFE1

Database References

UniProtID GeneID
Bos taurus A6QP15 507711
Mus musculus Q78SV3 76187
Western clawed frog Q6P371 394891
Pongo abelii Q5R5E1 100174480
Homo sapiens B4DTJ8 137872
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Protein Overview data has been sourced from Uniprot Consortium's databases under a Creative Commons Attribution-Commercial license. © 2017