HIBCH 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. Belongs to the enoyl-CoA hydratase/isomerase family. Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC; Hydrolase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine
Chromosomal Location of Human Ortholog: 1|1 C1.1
Cellular Component:  mitochondrion
Molecular Function:  3-hydroxyisobutyryl-CoA hydrolase activity; hydrolase activity
Biological Process:  branched-chain amino acid catabolic process; fatty acid beta-oxidation
Reference #:  Q8QZS1 (UniProtKB)
Alt. Names/Synonyms: 2610509I15Rik; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-coenzyme A hydrolase; AI648812; HIB-CoA hydrolase; Hibch; HIBYL-C; HIBYL-CoA-H; MGC31364
Gene Symbols: Hibch
Molecular weight: 43,038 Da
Basal Isoelectric point: 8.15  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene