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 3.1.2.4; Hydrolase; Mitochondrial; Other Amino Acids Metabolism - beta-alanine
Chromosomal Location of Human Ortholog: 2q32.2
Cellular Component:  mitochondrial matrix
Molecular Function:  3-hydroxyisobutyryl-CoA hydrolase activity
Biological Process:  branched-chain amino acid catabolic process; valine catabolic process
Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency
Reference #:  Q6NVY1 (UniProtKB)
Alt. Names/Synonyms: 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBCH; HIBYL-CoA-H
Gene Symbols: HIBCH
Molecular weight: 43,482 Da
Basal Isoelectric point: 8.38  Predict pI for various phosphorylation states
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HIBCH

Protein Structure Not Found.


Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein