3-hydroxyisobutyryl-CoA hydrolase
| 3-hydroxyisobutyryl-CoA hydrolase | |||||||||
|---|---|---|---|---|---|---|---|---|---|
 3-hydroxyisobutyryl-CoA hydrolase monomer, Human | |||||||||
| Identifiers | |||||||||
| EC no. | 3.1.2.4 | ||||||||
| CAS no. | 9025-88-1 | ||||||||
| Databases | |||||||||
| BRENDA | enzyme data | ||||||||
| ExPASy | NiceZyme view | ||||||||
| KEGG | enzyme entry | ||||||||
| MetaCyc | metabolic pathway | ||||||||
| Rhea | reactions | ||||||||
| PDB structures | RCSB PDB PDBe PDBsum | ||||||||
| Gene Ontology | AmiGO / QuickGO | ||||||||
| |||||||||
The enzyme 3-hydroxyisobutyryl-CoA hydrolase (EC 3.1.2.4) catalyzes the reaction
The enzyme characterised from pig heart and Phaeodactylum tricornutum hydrolyses 3-hydroxy-2-methylpropanoyl-CoA to coenzyme A and the acid, 3-hydroxyisobutyric acid, which forms the thioester in the starting material. The reaction is part of a sequence that breaks down the amino acids valine and isoleucine.[1][2] Deficiency of the enzyme in humans can cause a rare disease with three subtypes.[3][4]
This enzyme is a hydrolase, specifically one acting on thioester bonds. The systematic name is 3-hydroxy-2-methylpropanoyl-CoA hydrolase. Other names in common use include 3-hydroxy-isobutyryl CoA hydrolase, and HIB CoA deacylase.[5] 3-hydroxyisobutyryl-CoA hydrolase is encoded by HIBCH gene.[6]
References
[edit]- ↑ Rendina, George; Coon, Minor J. (1957). "Enzymatic hydrolysis of the coenzyme A thiol esters of β-hydroxypropionic and β-hydroxyisobutyric acids". Journal of Biological Chemistry. 225: 523–534. doi:10.1016/S0021-9258(18)64949-X. PMID 13457352.
- ↑ Pan, Yufang; Yang, Juan; Gong, Yangmin; Li, Xiaolong; Hu, Hanhua (2017). "3-Hydroxyisobutyryl-CoA hydrolase involved in isoleucine catabolism regulates triacylglycerol accumulation in Phaeodactylum tricornutum". Philosophical Transactions of the Royal Society B: Biological Sciences. 372 (1728). doi:10.1098/rstb.2016.0409. PMC 5516118. PMID 28717019.
- ↑ Yamada, Kenichiro; Naiki, Misako; Hoshino, Shin; et al. (2014). "Clinical and biochemical characterization of 3-hydroxyisobutyryl-CoA hydrolase (HIBCH) deficiency that causes Leigh-like disease and ketoacidosis". Molecular Genetics and Metabolism Reports. 1: 455–460. doi:10.1016/j.ymgmr.2014.10.003. PMC 5121361. PMID 27896122.
- ↑ Abdenur, J; Barrick, R. (2026-02-12). 3-Hydroxyisobutyryl-CoA Hydrolase Deficiency. Seattle: University of Washington.
- ↑ Enzyme 3.1.2.4 at KEGG Pathway Database.
- ↑ "OMIM Entry - * 610690 - 3-Hydroxyisobutyryl-CoA Hydrolase; HIBCH". www.omim.org. Retrieved 2017-11-20.
