3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; HIBYL-CoA-H; hib-coa hydrolase (hibch)

Function

• valine catabolism
• 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

More general terms

• mitochondrial protein
• hydrolase
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=26275
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:26275
• OMIM: https://mirror.omim.org/entry/250620
• OMIM: https://mirror.omim.org/entry/610690
• UniProt: http://www.uniprot.org/uniprot/Q6NVY1.html