UniProt ID: Q7ZV32 |
FUNCTION: Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3- methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta- hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism. {ECO:0000256|ARBA:ARBA00002768}. CATALYTIC ACTIVITY: Reaction=(3S)-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA; Xref=Rhea:RHEA:24404, ChEBI:CHEBI:13705, ChEBI:CHEBI:43074, ChEBI:CHEBI:57288; EC=4.1.3.4; Evidence={ECO:0000256|ARBA:ARBA00001651}; PATHWAY: Metabolic intermediate metabolism; (S)-3-hydroxy-3- methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3- methylglutaryl-CoA: step 1/1. {ECO:0000256|ARBA:ARBA00005143}. SUBUNIT: Homodimer; disulfide-linked. Can also form homotetramers. {ECO:0000256|ARBA:ARBA00011413}. SIMILARITY: Belongs to the HMG-CoA lyase family. {ECO:0000256|ARBA:ARBA00009405}. |
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