Reaction: HMGCLL1:Mg2+ cleaves bHMG-CoA to Ac-CoA and ACA
- in pathway: Synthesis of Ketone Bodies
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).
Reaction - small molecule participants:
Ac-CoA [cytosol]
ACA [cytosol]
bHMG-CoA [cytosol]
Reactome.org reaction link: R-HSA-6788597
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Reaction input - small molecules:
(3S)-3-hydroxy-3-methylglutaryl-CoA(5-)
Reaction output - small molecules:
acetyl-CoA(4-)
acetoacetate
Reactome.org link: R-HSA-6788597