Hydroxymethylglutaryl-CoA lyase, mitochondrial (IPR030020)

Short name: HL_mitochondrial

Overlapping homologous superfamilies

Family relationships



Hydroxymethylglutaryl-CoA lyase (HL) is a key enzyme in ketogenesis (ketone body formation) [PMID: 8566388]. It catalyse the final step in ketogenesis and leucine catabolism. There are two isoforms of the HL protein, mitochondrial and peroxisomal isoforms [PMID: 7527399]. Both isoforms are encoded by the same gene HMGCL and both exhibit lyase activity [PMID: 22847177]. The mitochondrial isoform contributes to ketone body production, while the function of the peroxisomal isoform is not clear [PMID: 22847177].

GO terms

Biological Process

GO:0046951 ketone body biosynthetic process

Molecular Function

GO:0004419 hydroxymethylglutaryl-CoA lyase activity

Cellular Component

GO:0005739 mitochondrion

Contributing signatures

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