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

Publication

PubMed ID:
8824301 15815621 15489334 19608861 21269460 24275569 25944712 17160907