EC - Carnosine synthase

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IntEnz Enzyme Nomenclature


Accepted name:
carnosine synthase
Other names:
carnosine synthetase
carnosine-anserine synthetase
carnosine-homocarnosine synthetase
homocarnosine-carnosine synthetase
Systematic name:
L-histidine:β-alanine ligase (AMP-forming)



This enzyme was thought to form AMP [1,2], but studies with highly purified enzyme proved that it forms ADP [4]. Carnosine is a dipeptide that is present at high concentrations in skeletal muscle and the olfactory bulb of vertebrates [3]. It is also found in the skeletal muscle of some invertebrates. The enzyme can also catalyse the formation of homocarnosine from 4-aminobutyrate and L-histidine, with much lower activity [4].

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ERGO , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0047730 , GO:0102102
CAS Registry Number: 9023-61-4


  1. Kalyankar, G.D. and Meister, A.
    Enzymatic synthesis of carnosine and related β-alanyl and γ-aminobutyryl peptides.
    J. Biol. Chem. 234: 3210-3218 (1959). [PMID: 14404206]
  2. Stenesh, J.J. and Winnick, T.
    Carnosine-anserine synthetase of muscle. 4. Partial purification of the enzyme and further studies of β-alanyl peptide synthesis.
    Biochem. J. 77: 575-581 (1960). [PMID: 16748858]
  3. Crush, K. G.
    Carnosine and related substances in animal tissues.
    Comp. Biochem. Physiol. 34: 3-30 (1970). [PMID: 4988625]
  4. Drozak, J., Veiga-da-Cunha, M., Vertommen, D., Stroobant, V., Van Schaftingen, E.
    Molecular identification of carnosine synthase as ATP-grasp domain-containing protein 1 (ATPGD1).
    J. Biol. Chem. 285: 9346-9356 (2010). [PMID: 20097752]

[EC created 1965, modified 2010]