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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 »

Target Id 1265
Nomenclature Propionyl-CoA carboxylase
Common abbreviation PCCA,PCCB
Subunits Propionyl-CoA carboxylase α subunit
Propionyl-CoA carboxylase β subunit
EC number
Endogenous substrates
Comment Propionyl-CoA carboxylase is able to function in both forward and reverse activity modes, as a ligase (carboxylase) or lyase (decarboxylase), respectively. A mRNA-nanoparticle (mRNA-3927) that encodes both the PCCA and PCCB subunits is in clinical development to replace the defective proteins in patients with the ultra-rare metabolic disease propionic acidaemia [3].

Enzymes and Subunits

PC (Pyruvate carboxylase) C Show summary »

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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Further reading

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How to cite this family page

Database page citation:

Carboxylases. Accessed on 14/07/2024. IUPHAR/BPS Guide to PHARMACOLOGY,

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie AA, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Davies JA et al. (2023) The Concise Guide to PHARMACOLOGY 2023/24: Enzymes. Br J Pharmacol. 180 Suppl 2:S289-373.