Enoyl-CoA hydratase/isomerase, HIBYL-CoA-H type (IPR032259)

Short name: HIBYL-CoA-H

Overlapping homologous superfamilies

Family relationships



The enoyl-CoA hydratase/isomerase family contains a diverse set of enzymes including: enoyl-CoA hydratase, napthoate synthase, carnitate racemase, 3-hydroxybutyryl-CoA dehydratase and dodecanoyl-CoA delta-isomerase. This entry represents a subset of the enoyl-CoA hydratase/isomerase family. Proteins in this entry includes 3-hydroxyisobutyryl-CoA hydrolases (HIBYL-CoA-H) from eukaryotes and their homologues from bacteria.

Human HIBYL-CoA-H is a mitochondrial enzyme that catalyses the fifth step in the valine catabolic pathway in eukaryotes, namely the conversion of 3-hydroxyisobutyryl-CoA to free 3-hydroxyisobutyrate [PMID: 8824301, PMID: 21104317]. It also hydrolyses 3-hydroxypropionyl-CoA, giving it a dual role in a secondary pathway of propionate metabolism [PMID: 26163321]. Deficiency of this enzyme is associated with Leigh-like disease [PMID: 25125611, PMID: 24299452].

GO terms

Biological Process

No terms assigned in this category.

Molecular Function

GO:0003860 3-hydroxyisobutyryl-CoA hydrolase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.