Hmdb loader
Identification
HMDB Protein ID HMDBP00379
Secondary Accession Numbers
  • 5616
  • HMDBP03243
  • HMDBP05449
Name 3-hydroxyacyl-CoA dehydrogenase type-2
Synonyms
  1. 17-beta-HSD 10
  2. 17-beta-hydroxysteroid dehydrogenase 10
  3. 3-hydroxy-2-methylbutyryl-CoA dehydrogenase
  4. 3-hydroxyacyl-CoA dehydrogenase type II
  5. Endoplasmic reticulum-associated amyloid beta-peptide-binding protein
  6. Mitochondrial RNase P protein 2
  7. Mitochondrial ribonuclease P protein 2
  8. Short-chain type dehydrogenase/reductase XH98G2
  9. Type II HADH
Gene Name HSD17B10
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific 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. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).
Pathways
  • 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
  • 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
  • 3-hydroxyisobutyric acid dehydrogenase deficiency
  • 3-hydroxyisobutyric aciduria
  • 3-Methylcrotonyl Coa Carboxylase Deficiency Type I
  • 3-Methylglutaconic Aciduria Type I
  • 3-Methylglutaconic Aciduria Type III
  • 3-Methylglutaconic Aciduria Type IV
  • Alzheimer disease
  • Beta-Ketothiolase Deficiency
  • Fatty Acid Elongation In Mitochondria
  • Isobutyryl-coa dehydrogenase deficiency
  • Isovaleric acidemia
  • Isovaleric Aciduria
  • Long-chain-3-hydroxyacyl-coa dehydrogenase deficiency (LCHAD)
  • Maple Syrup Urine Disease
  • Methylmalonate Semialdehyde Dehydrogenase Deficiency
  • Methylmalonic Aciduria
  • Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
  • Propionic Acidemia
  • Short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)
  • Valine, leucine and isoleucine degradation
  • Valine, leucine and isoleucine degradation
  • Valproic Acid Metabolism Pathway
Reactions
(S)-3-hydroxyacyl-CoA + NAD → 3-oxoacyl-CoA + NADH details
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH details
2-Methyl-3-hydroxybutyryl-CoA + NAD → 2-Methylacetoacetyl-CoA + NADH + Hydrogen Ion details
GO Classification
Biological Process
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
Leydig cell differentiation
cell aging
tRNA processing
protein homotetramerization
lipid metabolic process
Cellular Component
mitochondrial matrix
endoplasmic reticulum
plasma membrane
mitochondrial inner membrane
Function
binding
catalytic activity
oxidoreductase activity
Molecular Function
NAD binding
beta-amyloid binding
3-hydroxy-2-methylbutyryl-CoA dehydrogenase activity
acetoacetyl-CoA reductase activity
cholate 7-alpha-dehydrogenase activity
estradiol 17-beta-dehydrogenase activity
steroid binding
3-hydroxyacyl-CoA dehydrogenase activity
Process
metabolic process
physiological process
metabolism
oxidation reduction
Cellular Location
  1. Mitochondrion
  2. Secreted
Gene Properties
Chromosome Location X
Locus Xp11.2
SNPs HSD17B10
Gene Sequence
>786 bp
ATGGCAGCAGCGTGTCGGAGCGTGAAGGGCCTGGTGGCGGTAATAACCGGAGGAGCCTCG
GGCCTGGGCCTGGCCACGGCGGAGCGACTTGTGGGGCAGGGAGCCTCTGCTGTGCTTCTG
GACCTGCCCAACTCGGGTGGGGAGGCCCAAGCCAAGAAGTTAGGAAACAACTGCGTTTTC
GCCCCAGCCGACGTGACCTCTGAGAAGGATGTGCAAACAGCTCTGGCTCTAGCAAAAGGA
AAGTTTGGCCGTGTGGATGTAGCTGTCAACTGTGCAGGCATCGCGGTGGCTAGCAAGACG
TACAACTTAAAGAAGGGCCAGACCCATACCTTGGAAGACTTCCAGCGAGTTCTTGATGTG
AATCTCATGGGCACCTTCAATGTGATCCGCCTGGTGGCTGGTGAGATGGGCCAGAATGAA
CCAGACCAGGGAGGCCAACGTGGGGTCATCATCAACACTGCCAGTGTGGCTGCCTTCGAG
GGTCAGGTTGGACAAGCTGCATACTCTGCTTCCAAGGGGGGAATAGTGGGCATGACACTG
CCCATTGCTCGGGATCTGGCTCCCATAGGTATCCGGGTGATGACCATTGCCCCAGGTCTG
TTTGGCACCCCACTGCTGACCAGCCTCCCAGAGAAAGTGTGCAACTTCTTGGCCAGCCAA
GTGCCCTTCCCTAGCCGACTGGGTGACCCTGCTGAGTATGCTCACCTCGTACAGGCCATC
ATCGAGAACCCATTCCTCAATGGAGAGGTCATCCGGCTGGATGGGGCCATTCGTATGCAG
CCTTGA
Protein Properties
Number of Residues 261
Molecular Weight 25983.695
Theoretical pI 7.211
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>3-hydroxyacyl-CoA dehydrogenase type-2
MAAACRSVKGLVAVITGGASGLGLATAERLVGQGASAVLLDLPNSGGEAQAKKLGNNCVF
APADVTSEKDVQTALALAKGKFGRVDVAVNCAGIAVASKTYNLKKGQTHTLEDFQRVLDV
NLMGTFNVIRLVAGEMGQNEPDQGGQRGVIINTASVAAFEGQVGQAAYSASKGGIVGMTL
PIARDLAPIGIRVMTIAPGLFGTPLLTSLPEKVCNFLASQVPFPSRLGDPAEYAHLVQAI
IENPFLNGEVIRLDGAIRMQP
GenBank ID Protein 57210025
UniProtKB/Swiss-Prot ID Q99714
UniProtKB/Swiss-Prot Entry Name HCD2_HUMAN
PDB IDs
GenBank Gene ID U96132
GeneCard ID HSD17B10
GenAtlas ID HSD17B10
HGNC ID HGNC:4800
References
General References
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