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Proteins/UNIPROT:P35914
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..
Function from UniProtKB (P35914), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
1 row
129 statements
129 statements · 8 reference databases · Subject: HMGCL · Sources: Bgee, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
3 reactions
CHEBI:15467acetoacetate+CHEBI:15351
Catalysed by HMGCL
Reaction pageLeft to rightBalancedEC 4.1.3.4peroxisome
Human-GEM MAR01449CC BY 4.0Reference data
CHEBI:15467acetoacetate+CHEBI:15351
Reaction pageLeft to rightBalancedEC 4.1.3.4mitochondrial matrix
Human-GEM MAR01577CC BY 4.0Reference data
3 names
Resolves to: HMGCL
(3S)-3-hydroxy-3-methylglutaryl-CoAacetoacetate+acetyl-CoA
Reaction pageDirection not assertedBalanced
Rhea RHEA:24404CC BY 4.0Reference data