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

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

Stable Identifier

R-HSA-6788597

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

5/5

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

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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