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Proteins/UNIPROT:O00763
Mitochondrial enzyme that catalyzes the carboxylation of acetyl-CoA to malonyl-CoA and plays a central role in fatty acid metabolism. Catalyzes a 2 steps reaction starting with the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain followed by the transfer of the carboxyl group from carboxylated biotin to acetyl-CoA. Through the production of malonyl-CoA that allosterically inhibits carnitine palmitoyltransferase 1 at the mitochondria, negatively regulates fatty acid oxidation (By similarity). Together with its cytosolic isozyme ACACA, which is involved in de novo fatty acid biosynthesis, promotes lipid storage (By similarity)..
Function from UniProtKB (O00763), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
119 statements
119 statements · 7 reference databases · Subject: ACACB · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Reactome, UniProt, WikiPathways
3 reactions
CHEBI:15351+MAM01422mMAM01357m+malonyl-CoA
Catalysed by ACACB
Reaction pageLeft to rightBalance undeterminableEC 6.4.1.2mitochondrial matrix
Human-GEM MAR04295CC BY 4.0Reference data
MAM01357m+ATP+hydrogencarbonateADP+MAM01422m+proton+phosphate(3-)
Catalysed by ACACB
3 names
Resolves to: ACACB
Human-GEM MAR20112CC BY 4.0Reference data
hydrogencarbonate+acetyl-CoA+ATP(4-)malonyl-CoA+ADP(3-)+hydrogenphosphate+hydron
Reaction pageLeft to rightBalanced
Rhea RHEA:11308CC BY 4.0Reference data