KEGG   ENZYME: 6.3.2.11Help
Entry
EC 6.3.2.11                 Enzyme                                 
Name
carnosine synthase;
carnosine synthetase;
carnosine-anserine synthetase;
homocarnosine-carnosine synthetase;
carnosine-homocarnosine synthetase;
L-histidine:beta-alanine ligase (AMP-forming) (incorrect)
Class
Ligases;
Forming carbon-nitrogen bonds;
Acid-D-amino-acid ligases (peptide synthases)
BRITE hierarchy
Sysname
L-histidine:beta-alanine ligase (ADP-forming)
Reaction(IUBMB)
ATP + L-histidine + beta-alanine = ADP + phosphate + carnosine [RN:R01164]
Reaction(KEGG)
Substrate
ATP [CPD:C00002];
L-histidine [CPD:C00135];
beta-alanine [CPD:C00099]
Product
ADP [CPD:C00008];
phosphate [CPD:C00009];
carnosine [CPD:C00386]
Comment
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-aminobutanoate and L-histidine, with much lower activity [4].
Pathway
Arginine and proline metabolism
Histidine metabolism
beta-Alanine metabolism
Orthology
K14755  
carnosine synthase
Genes
HSA: 
57571(CARNS1)
PTR: 
451368(CARNS1)
PPS: 
100992842(CARNS1)
GGO: 
101133589(CARNS1)
PON: 
100433050(CARNS1)
MCC: 
MMU: 
107239(Carns1)
RNO: 
309150(RGD1308234)
CFA: 
483695(CARNS1)
AML: 
100470611(CARNS1)
FCA: 
101080661(CARNS1)
BTA: 
783525(CARNS1)
ECB: 
100059309(CARNS1)
MDO: 
100028997(CARNS1)
SHR: 
100934818(CARNS1)
OAA: 
100087096 100681134
GGA: 
100359387(CARNS1)
MGP: 
100548525
ACS: 
100557340
XTR: 
100487967
 » show all
Taxonomy
Reference
1  [PMID:14404206]
  Authors
KALYANKAR GD, MEISTER A.
  Title
Enzymatic synthesis of carnosine and related beta-alanyl and gamma-aminobutyryl peptides.
  Journal
J. Biol. Chem. 234 (1959) 3210-8.
  Organism
Gallus gallus [GN:gga]
Reference
2  [PMID:16748858]
  Authors
Stenesh JJ, Winnick T.
  Title
Carnosine-anserine synthetase of muscle. 4. Partial purification of the enzyme and further studies of beta-alanyl peptide synthesis.
  Journal
Biochem. J. 77 (1960) 575-81.
  Organism
Gallus gallus [GN:gga]
Reference
3  [PMID:4988625]
  Authors
Crush KG
  Title
Carnosine and related substances in animal tissues.
  Journal
Comp. Biochem. Physiol. 34 (1970) 3-30.
Reference
4  [PMID:20097752]
  Authors
Drozak J, Veiga-da-Cunha M, Vertommen D, Stroobant V, Van Schaftingen E
  Title
Molecular identification of carnosine synthase as ATP-grasp domain-containing protein 1 (ATPGD1).
  Journal
J. Biol. Chem. 285 (2010) 9346-56.
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
9023-61-4
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