3-hydroxyisobutyryl-CoA hydrolase

Symbol

HIBCH (may also be known as: None)

Organism

Human

Description

This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]

Orthologs

SymbolSpecies
CELE_F09F7.4 Caenorhabditis elegans
CG5044-RB Drosophila melanogaster
hibch Danio rerio
Hibch Mus musculus
Hibch Rattus norvegicus
EHD3 Saccharomyces cerevisiae
ehd3 Schizosaccharomyces pombe

Links to external resources

Changes associated with this gene

Identifier Name Type Tissues Organism Gene Data Actions
DAA3784 Gene expression of HIBCH decreases with age Molecular skin Human HIBCH —

GO Terms

GO IDGO TermGO Category
GO:0009083 branched chain family amino acid catabolic process biological_process
GO:0034641 cellular nitrogen compound metabolic process biological_process
GO:0044281 small molecule metabolic process biological_process
GO:0005739 mitochondrion cellular_component
GO:0005759 mitochondrial matrix cellular_component
GO:0003824 catalytic activity molecular_function
GO:0003860 3-hydroxyisobutyryl-CoA hydrolase activity molecular_function
GO:0016787 hydrolase activity molecular_function