3-hydroxy-3-methylglutaryl-CoA lyase like 1 (HMGCLL1)

3-hydroxy-3-methylglutaryl-CoA lyase like 1 (HMGCLL1) is a non-mitochondrial enzyme that catalyzes the cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key reaction in ketogenesis, enabling nonhepatic tissues to produce ketone bodies for energy. It is localized primarily in the cytosol and endoplasmic reticulum, is structurally and functionally related to the mitochondrial HMG-CoA lyase (HMGCL), but is genetically distinct and lacks the N-terminal mitochondrial targeting sequence. HMGCLL1 displays broad tissue expression with predicted enrichment in the brain and is notably detected in certain brain cancer cells such as glioblastoma, where it localizes to Golgi-derived vesicles. While its full physiological and clinical relevance remain incompletely defined, current evidence suggests it plays a supportive metabolic role in extramitochondrial ketone body biosynthesis.

Other names

3-hydroxy-3-methylglutaryl-CoA lyase, cytoplasmicHMGCL-like 1Endoplasmic reticulum 3-hydroxy-3-methylglutaryl-CoA lyaseer-cHLDKFZP434G1411bA418P12.1ERCHLEC 4.1.3.43-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1Probable 3-hydroxymethyl-3-methylglutaryl-CoA lyase 2Endoplasmic reticulum and cytosol HMG-CoA lyase