EC - Biotin carboxylase

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IntEnz Enzyme Nomenclature


Accepted name:
biotin carboxylase
Other names:
biotin carboxylase (component of acetyl CoA carboxylase)
accC (gene name)
biotin-carboxyl-carrier-protein:carbon-dioxide ligase (ADP-forming)
Systematic name:
[biotin carboxyl-carrier protein]-biotin-N6-L-lysine:hydrogencarbonate ligase (ADP-forming)



This enzyme, part of an acetyl-CoA carboxylase complex, acts on a biotin carboxyl-carrier protein (BCCP) that has been biotinylated by EC, biotin—[biotin carboxyl-carrier protein] ligase. In some organisms the enzyme is part of a multi-domain polypeptide that also includes the carrier protein (e.g. mycobacteria). Yet in other organisms (e.g. mammals) this activity is included in a single polypeptide that also catalyses the transfer of the carboxyl group from biotin to acetyl-CoA (see EC, acetyl-CoA carboxylase).

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Protein domains and families: PROSITE:PDOC50975
Structural data: CSA , EC2PDB
Gene Ontology: GO:0004075
CAS Registry Number: 9075-71-2
UniProtKB/Swiss-Prot: (31) [show] [UniProt]


  1. Dimroth, P., Guchhait, R.B., Stoll, E. and Lane, M.D.
    Enzymatic carboxylation of biotin: molecular and catalytic properties of a component enzyme of acetyl CoA carboxylase.
    Proc. Natl. Acad. Sci. USA 67 : 1353-1360 (1970). [PMID: 4922289]
  2. Norman, E., De Smet, K. A., Stoker, N. G., Ratledge, C., Wheeler, P. R., Dale, J. W.
    Lipid synthesis in mycobacteria: characterization of the biotin carboxyl carrier protein genes from Mycobacterium leprae and M. tuberculosis.
    J. Bacteriol. 176 : 2525-2531 (1994). [PMID: 7909542]
  3. Janiyani, K., Bordelon, T., Waldrop, G. L., Cronan, J. E.
    Function of Escherichia coli biotin carboxylase requires catalytic activity of both subunits of the homodimer.
    J. Biol. Chem. 276 : 29864-29870 (2001). [PMID: 11390406]
  4. Chou, C. Y., Yu, L. P., Tong, L.
    Crystal structure of biotin carboxylase in complex with substrates and implications for its catalytic mechanism.
    J. Biol. Chem. 284 : 11690-11697 (2009). [PMID: 19213731]
  5. Broussard, T. C., Pakhomova, S., Neau, D. B., Bonnot, R., Waldrop, G. L.
    Structural Analysis of Substrate, Reaction Intermediate, and Product Binding in Haemophilus influenzae Biotin Carboxylase.
    Biochemistry 54 : 3860-3870 (2015). [PMID: 26020841]

[EC created 1976, modified 2014, modified 2018]