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Carboxylases C

Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).


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The carboxylases allow the production of new carbon-carbon bonds by introducing HCO3- or CO2 into target molecules. Two groups of carboxylase activities, some of which are bidirectional, can be defined on the basis of the cofactor requirement, making use of biotin (EC 6.4.1.-) or vitamin K hydroquinone (EC 4.1.1.-).


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PCCA,PCCB (Propionyl-CoA carboxylase ) C Show summary »

Enzymes and Subunits

PC (Pyruvate carboxylase) C Show summary »

Target Id 1262
Nomenclature Pyruvate carboxylase
Common abbreviation PC
Previous and unofficial names PCB | Pcx | pyruvate carboxylase, mitochondrial | pyruvic carboxylase | Pc
Genes PC (Hs), Pcx (Mm), Pc (Rn)
Ensembl ID ENSG0000017359 (Hs), ENSMUSG00000024892 (Mm), ENSRNOG00000019372 (Rn)
UniProtKB AC P11498 (Hs), Q05920 (Mm), P52873 (Rn)
EC number
Endogenous substrates
pyruvic acid
oxalacetic acid

ACC1 (Acetyl-CoA carboxylase 1) C Show summary » More detailed page go icon to follow link

ACC2 (Acetyl-CoA carboxylase 2) C Show summary » More detailed page go icon to follow link

PCCA (Propionyl-CoA carboxylase α subunit) Show summary »

PCCB (Propionyl-CoA carboxylase β subunit) Show summary »

GGCX (γ-Glutamyl carboxylase) C Show summary » More detailed page go icon to follow link


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How to cite this family page

Database page citation:

Carboxylases. Accessed on 25/05/2022. IUPHAR/BPS Guide to PHARMACOLOGY,

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie A, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Pawson AJ, Sharman JL, Southan C, Davies JA; CGTP Collaborators. (2019) The Concise Guide to PHARMACOLOGY 2019/20: Enzymes. Br J Pharmacol. 176 Issue S1: S297-S396.