Names & Taxonomy
- Uniprot ID:
- Q6NVY1
- Entry Name:
- HIBCH_HUMAN
- Status:
- reviewed
- Protein Names:
- 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial (EC 3.1.2.4) (3-hydroxyisobutyryl-coenzyme A hydrolase) (HIB-CoA hydrolase) (HIBYL-CoA-H)
- Gene Names:
- HIBCH
- Gene Names Primary:
- HIBCH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 386
- Sequence:
- MGQREMWRLMSRFNAFKRTNTILHHLRMSKHTDAAEEVLLEKKGCTGVITLNRPKFLNALTLNMIRQIYPQLKKWEQDPETFLIIIKGAGGKAFCAGGDIRVISEAEKAKQKIAPVFFREEYMLNNAVGSCQKPYVALIHGITMGGGVGLSVHGQFRVATEKCLFAMPETAIGLFPDVGGGYFLPRLQGKLGYFLALTGFRLKGRDVYRAGIATHFVDSEKLAMLEEDLLALKSPSKENIASVLENYHTESKIDRDKSFILEEHMDKINSCFSANTVEEIIENLQQDGSSFALEQLKVINKMSPTSLKITLRQLMEGSSKTLQEVLTMEYRLSQACMRGHDFHEGVRAVLIDKDQSPKWKPADLKEVTEEDLNNHFKSLGSSDLKF
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
- Pathway:
- Amino-acid degradation; L-valine degradation.
- Catalytic Activity:
- 3-hydroxy-2-methylpropanoyl-CoA + H(2)O = CoA + 3-hydroxy-2-methylpropanoate.
- Gene Ontology Go:
- extracellular exosome
mitochondrial matrix
3-hydroxyisobutyryl-CoA hydrolase activity
hydro-lyase activity
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
small molecule metabolic process
valine catabolic process - Gene Ontology Biological Process:
- branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
small molecule metabolic process
valine catabolic process - Gene Ontology Molecular Function:
- 3-hydroxyisobutyryl-CoA hydrolase activity
hydro-lyase activity - Gene Ontology Cellular Component:
- extracellular exosome
mitochondrial matrix - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Branched-chain amino acid catabolism
Complete proteome
Disease mutation
Hydrolase
Mitochondrion
Polymorphism
Reference proteome
Transit peptide