3-hydroxyacyl-CoA dehydrogenase type-2 (O08756)

Uniprot ID O08756
Protein Name 3-hydroxyacyl-CoA dehydrogenase type-2
Gene Name Hsd17b10
Species Mus musculus (Mouse)
Signal peptide(a) N Secretome P(b) 0.0
Function Mitochondrial dehydrogenase that catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone (By similarity). Catalyzes the third step in the beta-oxidation of fatty acids (By similarity). Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids (By similarity). Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids (By similarity). By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD) (By similarity). Essential for structural and functional integrity of mitochondria (PubMed:20077426). .In addition to mitochondrial dehydrogenase activity, moonlights as a component of mitochondrial ribonuclease P, a complex that cleaves tRNA molecules in their 5'-ends. Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity. The MRPP1-MRPP2 subcomplex catalyzes the formation of N(1)-methylguanine and N(1)-methyladenine at position 9 (m1G9 and m1A9, respectively) in tRNAs; HSD17B10/MRPP2 acting as a non-catalytic subunit. The MRPP1-MRPP2 subcomplex also acts as a tRNA maturation platform: following 5'-end cleavage by the mitochondrial ribonuclease P complex, the MRPP1-MRPP2 subcomplex enhances the efficiency of 3'-processing catalyzed by ELAC2, retains the tRNA product after ELAC2 processing and presents the nascent tRNA to the mitochondrial CCA tRNA nucleotidyltransferase TRNT1 enzyme. {ECO:0000250|UniProtKB:Q99714}.
GO - Molecular function
  • 3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity : IEA:UniProtKB-EC
  • 3-hydroxyacyl-CoA dehydrogenase activity : ISS:UniProtKB
  • acetoacetyl-CoA reductase activity : ISO:MGI
  • amyloid-beta binding : ISO:MGI
  • estradiol 17-beta-dehydrogenase activity : ISO:MGI
  • estrogen receptor binding : ISO:MGI
  • identical protein binding : ISO:MGI
  • NAD binding : ISO:MGI
  • steroid binding : ISO:MGI
  • testosterone dehydrogenase [NAD(P)] activity : ISS:UniProtKB
  • tRNA binding : ISS:UniProtKB
GO - Biological process
  • mitochondrial tRNA 3'-end processing : ISS:UniProtKB
  • mitochondrial tRNA 5'-end processing : ISS:UniProtKB
  • mitochondrial tRNA methylation : ISS:UniProtKB
  • mitochondrion organization : ISO:MGI
  • protein homotetramerization : ISS:UniProtKB
(a) The Signal peptide D-score cutoff for "YES"(having signal peptide) is 0.45.
(b) Non-classically secreted proteins should obtain an NN-score(Neural Networks score) exceeding the normal threshold of 0.5, but not at the same time be predicted to contain a signal peptide.