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. 2013 Dec 17;170(8):1797–1867. doi: 10.1111/bph.12451
Nomenclature Pyruvate carboxylase Acetyl-CoA carboxylase 1 Acetyl-CoA carboxylase 2 Propionyl-CoA carboxylase γ-Glutamyl carboxylase
Common abbreviation PC ACC1 ACC2 GGCX
HGNC, UniProt PC, P11498 ACACA, Q13085 ACACB, O00763 GGCX, P38435
Subunits Propionyl-CoA carboxylase α subunit, Propionyl-CoA carboxylase β subunit
EC number 6.4.1.1 6.4.1.2 6.4.1.2 6.4.1.3 4.1.1.90
Endogenous substrates ATP, pyruvic acid ATP, acetyl CoA ATP, acetyl CoA ATP, propionyl-CoA glutamyl peptides
Products ADP, oxalacetic acid, PO34- malonyl-CoA, ADP, PO34- malonyl-CoA, ADP, PO34- ADP, methylmalonyl-CoA, PO34- carboxyglutamyl peptides
Cofactors biotin biotin biotin biotin NADPH, vitamin K hydroquinone
Selective inhibitors (pIC50) TOFA 38 TOFA 38
Comment Citrate and other dicarboxylic acids are allosteric activators of acetyl-CoA carboxylase Citrate and other dicarboxylic acids are allosteric activators of acetyl-CoA carboxylase Propionyl-CoA carboxylase is able to function in both forward and reverse activity modes, as a ligase (carboxylase) or lyase (decarboxylase), respectively Loss-of-function mutations in γ-glutamyl carboxylase are associated with clotting disorders