Summary

Name HMGCL
Full Name Hydroxymethylglutaryl-CoA lyase, mitochondrial
Synonyms HL, HMG-CoA lyase, EC 4.1.3.4, 3-hydroxy-3-methylglutarate-CoA lyase |
Primary ID P35914
Links - -
Type protein
Relations 3
Pathways Ketone body metabolism
Function Mitochondrial 3-hydroxy-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. 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. {ECO:0000269|PubMed:22847177, ECO:0000269|PubMed:22865860, ECO:0000269|PubMed:8566388}.

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Relations

Regulator Mechanism target score ℹ
+ HMGCLdown-regulates quantity img/direct_inhibition.png chemical modification (3S)-3-hydroxy-3-methylglutaryl-CoA
Publications: 1 Organism: Homo Sapiens
+ HMGCLup-regulates quantity img/direct-activation.png chemical modification acetoacetic acid
Publications: 1 Organism: Homo Sapiens
+ HMGCLup-regulates quantity img/direct-activation.png chemical modification acetyl-CoA(4-)
Publications: 1 Organism: Homo Sapiens