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3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIBYL-CoA-H; hib-coa hydrolase (hibch)
Function:
1) valine catabolism
2) hydrolyzes 3-hydroxyisobutyryl-CoA, isobutyryl-CoA & 3-hydroxypropanoyl-CoA
3-hydroxy-2-methylpropanoyl-CoA + H2O CoA + 3-hydroxy-2-methylpropanoate
Structure: belongs to the enoyl-CoA hydratase/isomerase family
Compartment: mitochondrion
Alternative splicing: named isoforms=2
Expression:
- highly expressed in liver & kidney
- also detected in heart, muscle & brain (at protein level)
- not detected in lung
Pathology:
- defects in hibch are the cause of methacrylic aciduria
Laboratory:
- 3-hydroxyisobutyryl-CoA hydrolase in fibroblasts
General
hydrolase
mitochondrial protein
phosphoprotein
Properties
SIZE: MW = 43 kD
entity length = 386 aa
COMPARTMENT: mitochondria
MOTIF: binding site
SITE: 121-121
FOR-BINDING-OF: Substrate
binding site
SITE: 146-146
FOR-BINDING-OF: Substrate; via amide nitrogen
binding site
SITE: 169-169
FOR-BINDING-OF: Substrate
binding site
SITE: 177-177
FOR-BINDING-OF: Substrate
active site
Ser phosphorylation site {S234}
Ser phosphorylation site {S356}
Database Correlations
OMIM correlations
MORBIDMAP 610690
UniProt Q6NVY1
Pfam PF16113
Entrez Gene 26275
Kegg hsa:26275
ENZYME 3.1.2.4
References
UniProt :accession Q6NVY1