3-hydroxyacyl-CoA dehydrogenase type-2 (Q99714)

Uniprot ID	Q99714
Protein Name	3-hydroxyacyl-CoA dehydrogenase type-2
Gene Name	HSD17B10
Species	Homo sapiens (Human)

Signal peptide^(a)	N	Secretome P^(b)	0.758
Function	Mitochondrial dehydrogenase that catalyzes the beta-oxidation at position 17 of androgens and estrogens and has 3-alpha-hydroxysteroid dehydrogenase activity with androsterone (PubMed:9553139, PubMed:23042678, PubMed:12917011, PubMed:18996107, PubMed:25925575, PubMed:28888424). Catalyzes the third step in the beta-oxidation of fatty acids (PubMed:9553139, PubMed:12917011, PubMed:18996107, PubMed:25925575, PubMed:28888424). Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids (PubMed:12917011). Also exhibits 20-beta-OH and 21-OH dehydrogenase activities with C21 steroids (PubMed:12917011). By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD) (PubMed:9338779). 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 (PubMed:18984158, PubMed:24549042, PubMed:25925575, PubMed:26950678, PubMed:28888424). 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 (PubMed:23042678, PubMed:29040705). 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 (PubMed:23042678, PubMed:25925575, PubMed:28888424). 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 (PubMed:29040705). {ECO:0000269\|PubMed:18984158, ECO:0000269\|PubMed:23042678, ECO:0000269\|PubMed:24549042, ECO:0000269\|PubMed:25925575, ECO:0000269\|PubMed:26950678, ECO:0000269\|PubMed:28888424, ECO:0000269\|PubMed:29040705}.
GO - Molecular function	3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity : IEA:UniProtKB-EC 3-hydroxyacyl-CoA dehydrogenase activity : IDA:UniProtKB cholate 7-alpha-dehydrogenase activity : TAS:ProtInc RNA binding : HDA:UniProtKB testosterone dehydrogenase [NAD(P)] activity : IDA:UniProtKB tRNA binding : IDA:UniProtKB
GO - Biological process	branched-chain amino acid catabolic process : TAS:Reactome lipid metabolic process : TAS:ProtInc mitochondrial tRNA 3'-end processing : IDA:UniProtKB mitochondrial tRNA 5'-end processing : IDA:UniProtKB mitochondrial tRNA methylation : IDA:UniProtKB mitochondrial tRNA processing : TAS:Reactome mitochondrion organization : IMP:UniProtKB protein homotetramerization : IDA:UniProtKB

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^{(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.}