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Description

3-hydroxyacyl-CoA dehydrogenase type-2 (UniProt: Q99714, also known as 17-beta-hydroxysteroid dehydrogenase 10, 17-beta-HSD 10, 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type II, Endoplasmic reticulum-associated amyloid beta-peptide-binding protein, Mitochondrial ribonuclease P protein 2, Mitochondrial RNase P protein 2, Short chain dehydrogenase/ reductase family 5C member 1, Short-chain type dehydrogenase/reductase XH98G2, Type II HADH) is encoded by the HSD17B10 (also known as ERAB, HADH2, MRPP2, SCHAD, SDR5C1, XH98G2) gene (Gene ID: 3028) in human. 3-hydroxyacyl-CoA dehydrogenase type-2 is a part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5&prime,-ends. It functions in mitochondrial tRNA maturation. It catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone. It catalyzes the third step in the beta-oxidation of fatty acids and also carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. With C21 steroids it exhibits 20-beta-OH and 21-OH dehydrogenase activities. May contribute to neuronal dysfunction associated with Alzheimer disease (AD) via interactions with intracellular amyloid-beta peptides. 3-hydroxyacyl-CoA dehydrogenase type-2 is ubiquitously expressed in normal tissues, but is overexpressed in neurons affected in AD.