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Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/TRMT10C, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. Catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).
Gene Name: | hydroxysteroid (17-beta) dehydrogenase 10 |
Synonyms: | HSD17B10, 17-beta-HSD 10, 17b-HSD10, CAMR, DUPXp11.22, ABAD, ERAB, HADH2, MHBD, MRPP2, MRX17, SCHAD, SDR5C1, MRX31, MRXS10, Type II HADH, HCD2, XH98G2 |
Target Sequences: | NM_004493 NP_004484.1 Q99714 |
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