LMPD Database

LMP012745

UniProt Annotations

Entry Information
Gene Name3-hydroxymethyl-3-methylglutaryl-CoA lyase
Protein EntryHMGCL_RAT
UniProt IDP97519
SpeciesRat
Comments
Comment typeDescription
Catalytic Activity(S)-3-hydroxy-3-methylglutaryl-CoA = acetyl- CoA + acetoacetate
FunctionKey enzyme in ketogenesis (ketone body formation). 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 (By similarity)
PathwayMetabolic intermediate metabolism; (S)-3-hydroxy-3- methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3- methylglutaryl-CoA: step 1/1.
SimilarityBelongs to the HMG-CoA lyase family
Subcellular LocationMitochondrion matrix . Peroxisome . Note=Unprocessed form is peroxisomal
SubunitHomodimer; disulfide-linked. Can also form homotetramers
Tissue SpecificityIn suckling rat, highest levels in liver and in intestine. Lower levels in heart, kidney and cerebellum. Weak expression in brain cortex, medulla and midbrain. Levels decrease slightly during weaning