Names & Taxonomy
- Uniprot ID:
- Q99714
- Entry Name:
- HCD2_HUMAN
- Status:
- reviewed
- Protein Names:
- 3-hydroxyacyl-CoA dehydrogenase type-2 (EC 1.1.1.35) (17-beta-hydroxysteroid dehydrogenase 10) (17-beta-HSD 10) (EC 1.1.1.51) (3-hydroxy-2-methylbutyryl-CoA dehydrogenase) (EC 1.1.1.178) (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)
- Gene Names:
- HSD17B10 ERAB HADH2 MRPP2 SCHAD SDR5C1 XH98G2
- Gene Names Primary:
- HSD17B10
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 261
- Sequence:
- MAAACRSVKGLVAVITGGASGLGLATAERLVGQGASAVLLDLPNSGGEAQAKKLGNNCVFAPADVTSEKDVQTALALAKGKFGRVDVAVNCAGIAVASKTYNLKKGQTHTLEDFQRVLDVNLMGTFNVIRLVAGEMGQNEPDQGGQRGVIINTASVAAFEGQVGQAAYSASKGGIVGMTLPIARDLAPIGIRVMTIAPGLFGTPLLTSLPEKVCNFLASQVPFPSRLGDPAEYAHLVQAIIENPFLNGEVIRLDGAIRMQP
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- 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).
- Catalytic Activity:
- (S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.; (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA + NAD(+) = 2-methylacetoacetyl-CoA + NADH.; Testosterone + NAD(P)(+) = androst-4-ene-3,17-dione + NAD(P)H.
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=25.7 uM for acetoacetyl-CoA (in the presence of 0.2 mM NADH, at pH 7.0 and 25 degrees Celsius)
- Active Site:
- ACT_SITE 168 168 Proton acceptor.
- Gene Ontology Go:
- cytoplasm
mitochondrial matrix
mitochondrion
plasma membrane
3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity
3-hydroxyacyl-CoA dehydrogenase activity
acetoacetyl-CoA reductase activity
beta-amyloid binding
cholate 7-alpha-dehydrogenase activity
estradiol 17-beta-dehydrogenase activity
NAD binding
poly(A) RNA binding
steroid binding
testosterone dehydrogenase activity
branched-chain amino acid catabolic process
cell aging
cellular nitrogen compound metabolic process
gene expression
Leydig cell differentiation
lipid metabolic process
mitochondrial tRNA modification
mitochondrial tRNA processing
protein homotetramerization
small molecule metabolic process
tRNA processing - Gene Ontology Biological Process:
- branched-chain amino acid catabolic process
cell aging
cellular nitrogen compound metabolic process
gene expression
Leydig cell differentiation
lipid metabolic process
mitochondrial tRNA modification
mitochondrial tRNA processing
protein homotetramerization
small molecule metabolic process
tRNA processing - Gene Ontology Molecular Function:
- 3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity
3-hydroxyacyl-CoA dehydrogenase activity
acetoacetyl-CoA reductase activity
beta-amyloid binding
cholate 7-alpha-dehydrogenase activity
estradiol 17-beta-dehydrogenase activity
NAD binding
poly(A) RNA binding
steroid binding
testosterone dehydrogenase activity - Gene Ontology Cellular Component:
- cytoplasm
mitochondrial matrix
mitochondrion
plasma membrane - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Chromosomal rearrangement
Complete proteome
Disease mutation
Mental retardation
Mitochondrion
NAD
Oxidoreductase
Reference proteome
tRNA processing - Interacts With:
- P05067; Q7L0Y3