HMGCLL1:Mg2+ cleaves bHMG-CoA to Ac-CoA and ACA

Stable Identifier
Homo sapiens
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3-hydroxy-3-methylglutaryl CoA lyase (HMGCL) catalyses the cleavage of beta-hydroxy-3-methylglutaryl CoA (bHMG-CoA) to acetoacetate (ACA) and acetyl-CoA (Ac-CoA). This reaction is the common final step in ketogenesis and leucine catabolism. HMGCL isoforms are usually located to the mitochondrial matrix or peroxisomes. The mitochondrial isoform contributes to ketone body production whereas the function of the peroxisomal form is unclear. Another isoform has recently been discovered, (HMGCLL1) which is expressed mainly in liver, lung, kidney and brain and has a dual location of ER and cytosolic cellular compartments. The metabolic function of HMGCLL1 is unknown but could provide support for energy production in the tissues mentioned above (Montgomery et al. 2012, Arnedo et al. 2012).

Literature References
PubMed ID Title Journal Year
22847177 Characterization of a novel HMG-CoA lyase enzyme with a dual location in endoplasmic reticulum and cytosol

López-Viñas, E, Gil-Rodríguez, MC, Casals, N, Pie, J, Hegardt, FG, Casale, CH, Gomez-Puertas, P, Menao, S, Teresa-Rodrigo, ME, Puisac, B, Arnedo, M

J. Lipid Res. 2012
22865860 Identification and characterization of an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase

Watkins, PA, Miziorko, HM, Pei, Z, Montgomery, C

J. Biol. Chem. 2012
Catalyst Activity

hydroxymethylglutaryl-CoA lyase activity of HMGCLL1:Mg2+ [cytosol]

Orthologous Events
Cross References
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