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].
History
EC 6.3.2.11 created 1965, modified 2010
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)
NLE: 
100586652(CARNS1)
MCC: 
MCF: 
102140146(CARNS1)
CJC: 
100396814(CARNS1)
MMU: 
107239(Carns1)
RNO: 
309150(Carns1)
CGE: 
100765077(Carns1)
HGL: 
101703269(Carns1)
TUP: 
102476516(CARNS1)
CFA: 
483695(CARNS1)
AML: 
100470611(CARNS1)
UMR: 
103679612(CARNS1)
FCA: 
101080661(CARNS1)
PTG: 
102953183(CARNS1)
BTA: 
783525(CARNS1)
BOM: 
102288119(CARNS1)
PHD: 
102339857(CARNS1)
CHX: 
102182385(CARNS1)
OAS: 
101109312(CARNS1)
SSC: 
100512885(CARNS1)
CFR: 
BACU: 
103008596(CARNS1)
LVE: 
103080369(CARNS1)
ECB: 
100059309(CARNS1)
MYB: 
102244811(CARNS1)
MYD: 
102764108(CARNS1)
PALE: 
102891454(CARNS1)
MDO: 
100028997(CARNS1)
SHR: 
100934818(CARNS1)
OAA: 
GGA: 
100359387(CARNS1)
MGP: 
FAB: 
101810237(CARNS1)
PHI: 
102100376(CARNS1)
FPG: 
101914136(CARNS1)
FCH: 
102053346(CARNS1)
CLV: 
102090970(CARNS1)
ASN: 
102369760(CARNS1)
AMJ: 
102565182(CARNS1)
PSS: 
CMY: 
102932637(CARNS1)
ACS: 
100557340(carns1)
PBI: 
103057026(CARNS1)
XTR: 
CRE: 
VCN: 
CSL: 
CVR: 
CME: 
GSL: 
CCP: 
 » 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.
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.
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.
  Sequence
[gga:100359387] [hsa:57571] [mmu:107239]
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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