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 |