KEGG   ORTHOLOGY: K14755
Entry
K14755                      KO                                     
Symbol
CRNS1, ATPGD1
Name
carnosine synthase [EC:6.3.2.11]
Pathway
map00330  Arginine and proline metabolism
map00340  Histidine metabolism
map00410  beta-Alanine metabolism
map01100  Metabolic pathways
Reaction
R00910  L-lysine:beta-alanine ligase (ADP-forming)
R00912  L-arginine:beta-alanine ligase (ADP-forming)
R01164  L-histidine:beta-alanine ligase (ADP-forming)
R01991  L-histidine:4-aminobutanoate ligase (ADP-forming)
R03286  N(pi)-methyl-L-histidine:beta-alanine ligase (ADP-forming)
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09105 Amino acid metabolism
   00330 Arginine and proline metabolism
    K14755  CRNS1, ATPGD1; carnosine synthase
   00340 Histidine metabolism
    K14755  CRNS1, ATPGD1; carnosine synthase
  09106 Metabolism of other amino acids
   00410 beta-Alanine metabolism
    K14755  CRNS1, ATPGD1; carnosine synthase
Enzymes [BR:ko01000]
 6. Ligases
  6.3  Forming carbon-nitrogen bonds
   6.3.2  Acid-D-amino-acid ligases (peptide synthases)
    6.3.2.11  carnosine synthase
     K14755  CRNS1, ATPGD1; carnosine synthase
Other DBs
GO: 0047730
Genes
HSA: 57571(CARNS1)
PTR: 451368(CARNS1)
PPS: 100992842(CARNS1)
GGO: 101133589(CARNS1)
PON: 100433050(CARNS1)
PPYG: 129007092(CARNS1)
NLE: 100586652(CARNS1)
HMH: 116469075(CARNS1)
SSYN: 129478259(CARNS1)
MCC: 712485(CARNS1)
MCF: 102140146(CARNS1)
MTHB: 126935432
MNI: 105469678(CARNS1)
CSAB: 103217912(CARNS1)
CATY: 105577355(CARNS1)
PANU: 101012781(CARNS1)
TGE: 112606990(CARNS1)
RRO: 104664741(CARNS1)
RBB: 108518609(CARNS1)
TFN: 117080245(CARNS1)
PTEH: 111522251(CARNS1)
CANG: 105512324(CARNS1)
CJC: 100396814(CARNS1)
SBQ: 101048183(CARNS1)
CIMI: 108310358(CARNS1)
ANAN: 105717659(CARNS1)
MMUR: 105867471(CARNS1)
LCAT: 123642850(CARNS1)
PCOQ: 105827361(CARNS1)
OGA: 100941290(CARNS1)
MMU: 107239(Carns1)
MCAL: 110286079(Carns1)
MPAH: 110336527(Carns1)
RNO: 309150(Carns1)
MCOC: 116103323(Carns1)
ANU: 117713991(Carns1)
MUN: 110560365(Carns1)
CGE: 100765077(Carns1)
MAUA: 101828407(Carns1)
PROB: 127228637(Carns1)
PLEU: 114685990(Carns1)
MORG: 121453760(Carns1)
MFOT: 126498508
AAMP: 119805188(Carns1)
NGI: 103725587(Carns1)
HGL: 101703269(Carns1)
CPOC: 100733118(Carns1)
CCAN: 109688626(Carns1)
DORD: 105989174(Carns1)
DSP: 122105364(Carns1)
PLOP: 125362629(Carns1)
NCAR: 124958804
MMMA: 107156877(Carns1)
OCU: 127484699
OPI: 101525872(CARNS1)
TUP: 102476516(CARNS1)
GVR: 103589997(CARNS1)
CFA: 483695(CARNS1)
CLUD: 112659426(CARNS1)
VVP: 112924184(CARNS1)
VLG: 121472179(CARNS1)
NPO: 129503142(CARNS1)
AML: 100470611(CARNS1)
UMR: 103679612(CARNS1)
UAH: 113244030(CARNS1)
UAR: 123778450(CARNS1)
ELK: 111149703
LLV: 125079633
MPUF: 101671143(CARNS1)
MNP: 132017865(CARNS1)
MLK: 131816424(CARNS1)
NVS: 122913181(CARNS1)
ORO: 101382286(CARNS1)
EJU: 114220188(CARNS1)
ZCA: 113914532(CARNS1)
MLX: 118016128(CARNS1)
NSU: 110576037(CARNS1)
FCA: 101080661(CARNS1)
PYU: 121023681(CARNS1)
PCOO: 112851966(CARNS1)
PBG: 122484050(CARNS1)
PVIV: 125147131(CARNS1)
LRUF: 124506130
PTG: 102953183(CARNS1)
PPAD: 109246848(CARNS1)
PUC: 125930596
AJU: 106971104
HHV: 120233868(CARNS1)
BTA: 783525(CARNS1)
BOM: 102288119(CARNS1)
BIU: 109554139(CARNS1)
BBUB: 102414079(CARNS1)
BBIS: 104987068(CARNS1)
CHX: 102182385(CARNS1)
OAS: 101109312(CARNS1)
BTAX: 128068706(CARNS1)
CSUM: 138084384(CARNS1)
ODA: 120874774(CARNS1)
CCAD: 122430820(CARNS1)
MREE: 136169796(CARNS1)
MBEZ: 129548693(CARNS1)
SSC: 100512885(CARNS1)
CFR: 102520872(CARNS1)
CBAI: 105070428(CARNS1)
CDK: 105104426(CARNS1)
VPC: 102536997(CARNS1)
BACU: 103008596(CARNS1)
BMUS: 118898756(CARNS1)
LVE: 103080369(CARNS1)
OOR: 101285110(CARNS1)
DLE: 111183670(CARNS1)
PCAD: 102981468
PSIU: 116757789(RPS6KB2)
NASI: 112402099(CARNS1)
ECB: 100059309(CARNS1)
EPZ: 103560159(CARNS1)
EAI: 106833657(CARNS1)
MYB: 102244811(CARNS1)
MYD: 102764108(CARNS1)
MMYO: 118665217(CARNS1)
MLF: 102436930(CARNS1)
MDT: 132241332(CARNS1)
PKL: 118706963(CARNS1)
EFUS: 103290925(CARNS1)
MNA: 107537447(CARNS1)
DRO: 112317161(CARNS1)
SHON: 118991816(CARNS1)
AJM: 119041869(CARNS1)
PDIC: 114500896(CARNS1)
PHAS: 123829616(CARNS1)
MMF: 118628350(CARNS1)
PPAM: 129068359(CARNS1)
HAI: 109384218(CARNS1)
RFQ: 117029820(CARNS1)
PALE: 102891454(CARNS1)
PGIG: 120584752(CARNS1)
PVP: 105306549(CARNS1)
RAY: 107503705(CARNS1)
MJV: 108393146(CARNS1)
TOD: 119249911(CARNS1)
SARA: 101549931(CARNS1)
SETR: 126018368(CARNS1)
TMU: 101343802
ETF: 101647156(CARNS1)
DNM: 101445662(CARNS1)
MDO: 100028997(CARNS1)
GAS: 123252078(CARNS1)
SHR: 100934818(CARNS1)
AFZ: 127541283
PCW: 110195424(CARNS1)
TVP: 118855561(CARNS1)
PBRV: 138161641(CARNS1)
OAA: 100681134(CARNS1)
GGA: 100359387(CARNS1)
PCOC: 116243023(CARNS1)
MGP: 100548525(CARNS1)
CJO: 107314238(CARNS1)
TPAI: 128086778(CARNS1)
LMUT: 125694971(CARNS1)
NMEL: 110390570
APLA: 119717127(CARNS1)
ACYG: 106048295(CARNS1)
CATA: 118244760(CARNS1)
AFUL: 116490065(CARNS1)
TGU: 115495355(CARNS1)
LSR: 110485040(CARNS1)
PMOA: 120499578(CARNS1)
PRUF: 121354246 121355168(CARNS1)
FAB: 101810237(CARNS1)
OMA: 130253395(CARNS1)
PHI: 102100376(CARNS1)
PMAJ: 107205283(CARNS1)
CCW: 120410069(CARNS1)
ETL: 114063334(CARNS1)
ZLE: 135448982(CARNS1)
SVG: 106854670(CARNS1)
MMEA: 130580074(CARNS1)
HRT: 120754252(CARNS1)
SATI: 136362105(CARNS1)
FPG: 106112844(CARNS1)
FCH: 102053346(CARNS1)
SHAB: 115607024(CARNS1)
TALA: 116962730(CARNS1)
ACHC: 115337544(CARNS1)
AGEN: 126047496
PPUS: 135186050(CARNS1)
CLV: 102090970(CARNS1)
CUCA: 104063361(CARNS1)
RTD: 128909665(CARNS1)
AROW: 112963283(CARNS1)
NPD: 112957577(CARNS1)
DNE: 112988613(CARNS1)
ASN: 102369760(CARNS1)
AMJ: 102565182(CARNS1)
CPOO: 109324621(CARNS1)
PSS: 102451775(CARNS1)
CMY: 102932637(CARNS1)
CCAY: 125638658(CARNS1)
DCC: 119857796(CARNS1)
CPIC: 101949086(CARNS1)
TST: 117876023(CARNS1)
CABI: 116833424(CARNS1)
MRV: 120393419(CARNS1)
ACS: 100557340(carns1)
ASAO: 132773259(CARNS1)
PVT: 110085648(CARNS1)
SUND: 121919270(CARNS1)
PBI: 103057026(CARNS1)
PMUR: 107293631(CARNS1)
CTIG: 120306944(CARNS1)
TSR: 106538898(CARNS1)
PGUT: 117675337(CARNS1)
PTEX: 113441114(CARNS1)
NSS: 113410868(CARNS1)
VKO: 123027364(CARNS1)
PMUA: 114602074(CARNS1)
PRAF: 128419314(CARNS1)
ZVI: 118090264(CARNS1)
HCG: 128341125(CARNS1)
GJA: 107121294(CARNS1)
STOW: 125425978(CARNS1)
EMC: 129323876(CARNS1)
XLA: 108696326(carns1.L)
XTR: 100487967(carns1)
NPR: 108796244
RTEM: 120909145(CARNS1)
BBUF: 121003071(CARNS1)
BGAR: 122940634(CARNS1)
MUO: 115456520(CARNS1)
GSH: 117348214(CARNS1)
SASA: 106611885
STRU: 115205380
SALP: 112069063
CCLU: 121579852
ELS: 105025204(carns1)
SFM: 108935907(carns1)
AANG: 118231339
LOC: 102683811 102697335(carns1)
PSEX: 120538030
PMRN: 116936845(CARNS1)
PCAN: 112554628
MYI: 110448302
PMAX: 117317717
MCAF: 127717988
LAK: 106157795
 » show all
Reference
  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 285:9346-56 (2010)
DOI:10.1074/jbc.M109.095505
  Sequence
[gga:100359387] [hsa:57571] [mmu:107239]
LinkDB

KEGG   ENZYME: 6.3.2.11
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)
Sysname
L-histidine:beta-alanine ligase (ADP-forming)
Reaction(IUBMB)
ATP + L-histidine + beta-alanine = ADP + phosphate + carnosine [RN:R01164]
Reaction(KEGG)
R01164;
(other) R00910 R00912 R01991 R03286
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
ec00330  Arginine and proline metabolism
ec00340  Histidine metabolism
ec00410  beta-Alanine metabolism
ec01100  Metabolic pathways
Orthology
K14755  carnosine synthase
Genes
HSA57571(CARNS1)
PTR451368(CARNS1)
PPS100992842(CARNS1)
GGO101133589(CARNS1)
PON100433050(CARNS1)
PPYG129007092(CARNS1)
NLE100586652(CARNS1)
HMH116469075(CARNS1)
SSYN129478259(CARNS1)
MCC712485(CARNS1)
MCF102140146(CARNS1)
MTHB126935432
MNI105469678(CARNS1)
CSAB103217912(CARNS1)
CATY105577355(CARNS1)
PANU101012781(CARNS1)
TGE112606990(CARNS1)
RRO104664741(CARNS1)
RBB108518609(CARNS1)
TFN117080245(CARNS1)
PTEH111522251(CARNS1)
CANG105512324(CARNS1)
CJC100396814(CARNS1)
SBQ101048183(CARNS1)
CIMI108310358(CARNS1)
ANAN105717659(CARNS1)
MMUR105867471(CARNS1)
LCAT123642850(CARNS1)
PCOQ105827361(CARNS1)
OGA100941290(CARNS1)
MMU107239(Carns1)
MCAL110286079(Carns1)
MPAH110336527(Carns1)
RNO309150(Carns1)
MCOC116103323(Carns1)
ANU117713991(Carns1)
MUN110560365(Carns1)
CGE100765077(Carns1)
MAUA101828407(Carns1)
PROB127228637(Carns1)
PLEU114685990(Carns1)
MORG121453760(Carns1)
MFOT126498508
AAMP119805188(Carns1)
NGI103725587(Carns1)
HGL101703269(Carns1)
CPOC100733118(Carns1)
CCAN109688626(Carns1)
DORD105989174(Carns1)
DSP122105364(Carns1)
PLOP125362629(Carns1)
NCAR124958804
MMMA107156877(Carns1)
OCU127484699
OPI101525872(CARNS1)
TUP102476516(CARNS1)
GVR103589997(CARNS1)
CFA483695(CARNS1)
CLUD112659426(CARNS1)
VVP112924184(CARNS1)
VLG121472179(CARNS1)
NPO129503142(CARNS1)
AML100470611(CARNS1)
UMR103679612(CARNS1)
UAH113244030(CARNS1)
UAR123778450(CARNS1)
ELK111149703
LLV125079633
MPUF101671143(CARNS1)
MNP132017865(CARNS1)
MLK131816424(CARNS1)
NVS122913181(CARNS1)
ORO101382286(CARNS1)
EJU114220188(CARNS1)
ZCA113914532(CARNS1)
MLX118016128(CARNS1)
NSU110576037(CARNS1)
FCA101080661(CARNS1)
PYU121023681(CARNS1)
PCOO112851966(CARNS1)
PBG122484050(CARNS1)
PVIV125147131(CARNS1)
LRUF124506130
PTG102953183(CARNS1)
PPAD109246848(CARNS1)
PUC125930596
AJU106971104
HHV120233868(CARNS1)
BTA783525(CARNS1)
BOM102288119(CARNS1)
BIU109554139(CARNS1)
BBUB102414079(CARNS1)
BBIS104987068(CARNS1)
CHX102182385(CARNS1)
OAS101109312(CARNS1)
BTAX128068706(CARNS1)
CSUM138084384(CARNS1)
ODA120874774(CARNS1)
CCAD122430820(CARNS1)
MREE136169796(CARNS1)
MBEZ129548693(CARNS1)
SSC100512885(CARNS1)
CFR102520872(CARNS1)
CBAI105070428(CARNS1)
CDK105104426(CARNS1)
VPC102536997(CARNS1)
BACU103008596(CARNS1)
BMUS118898756(CARNS1)
LVE103080369(CARNS1)
OOR101285110(CARNS1)
DLE111183670(CARNS1)
PCAD102981468
PSIU116757789(RPS6KB2)
NASI112402099(CARNS1)
ECB100059309(CARNS1)
EPZ103560159(CARNS1)
EAI106833657(CARNS1)
MYB102244811(CARNS1)
MYD102764108(CARNS1)
MMYO118665217(CARNS1)
MLF102436930(CARNS1)
MDT132241332(CARNS1)
PKL118706963(CARNS1)
EFUS103290925(CARNS1)
MNA107537447(CARNS1)
DRO112317161(CARNS1)
SHON118991816(CARNS1)
AJM119041869(CARNS1)
PDIC114500896(CARNS1)
PHAS123829616(CARNS1)
MMF118628350(CARNS1)
PPAM129068359(CARNS1)
HAI109384218(CARNS1)
RFQ117029820(CARNS1)
PALE102891454(CARNS1)
PGIG120584752(CARNS1)
PVP105306549(CARNS1)
RAY107503705(CARNS1)
MJV108393146(CARNS1)
TOD119249911(CARNS1)
SARA101549931(CARNS1)
SETR126018368(CARNS1)
TMU101343802
ETF101647156(CARNS1)
DNM101445662(CARNS1)
MDO100028997(CARNS1)
GAS123252078(CARNS1)
SHR100934818(CARNS1)
AFZ127541283
PCW110195424(CARNS1)
TVP118855561(CARNS1)
PBRV138161641(CARNS1)
OAA100681134(CARNS1)
GGA100359387(CARNS1)
PCOC116243023(CARNS1)
MGP100548525(CARNS1)
CJO107314238(CARNS1)
TPAI128086778(CARNS1)
LMUT125694971(CARNS1)
NMEL110390570
APLA119717127(CARNS1)
ACYG106048295(CARNS1)
CATA118244760(CARNS1)
AFUL116490065(CARNS1)
TGU115495355(CARNS1)
LSR110485040(CARNS1)
PMOA120499578(CARNS1)
OTC121339383 121339658
PRUF121354246 121355168(CARNS1)
FAB101810237(CARNS1)
OMA130253395(CARNS1)
PHI102100376(CARNS1)
PMAJ107205283(CARNS1)
CCW120410069(CARNS1)
ETL114063334(CARNS1)
ZLE135448982(CARNS1)
SVG106854670(CARNS1)
MMEA130580074(CARNS1)
HRT120754252(CARNS1)
SATI136362105(CARNS1)
FPG106112844(CARNS1)
FCH102053346(CARNS1)
SHAB115607024(CARNS1)
TALA116962730(CARNS1)
ACHC115337544(CARNS1)
AGEN126047496
PPUS135186050(CARNS1)
CLV102090970(CARNS1)
CUCA104063361(CARNS1)
RTD128909665(CARNS1)
AROW112963283(CARNS1)
NPD112957577(CARNS1)
DNE112988613(CARNS1)
ASN102369760(CARNS1)
AMJ102565182(CARNS1)
CPOO109324621(CARNS1)
PSS102451775(CARNS1)
CMY102932637(CARNS1)
CCAY125638658(CARNS1)
DCC119857796(CARNS1)
CPIC101949086(CARNS1)
TST117876023(CARNS1)
CABI116833424(CARNS1)
MRV120393419(CARNS1)
ACS100557340(carns1)
ASAO132773259(CARNS1)
PVT110085648(CARNS1)
SUND121919270(CARNS1)
PBI103057026(CARNS1)
PMUR107293631(CARNS1)
CTIG120306944(CARNS1)
TSR106538898(CARNS1)
PGUT117675337(CARNS1)
PTEX113441114(CARNS1)
NSS113410868(CARNS1)
VKO123027364(CARNS1)
PMUA114602074(CARNS1)
PRAF128419314(CARNS1)
ZVI118090264(CARNS1)
HCG128341125(CARNS1)
GJA107121294(CARNS1)
STOW125425978(CARNS1)
EMC129323876(CARNS1)
XLA108696326(carns1.L)
XTR100487967(carns1)
NPR108796244
RTEM120909145(CARNS1)
BBUF121003071(CARNS1)
BGAR122940634(CARNS1)
MUO115456520(CARNS1)
GSH117348214(CARNS1)
CHAR105912822 116224073
SASA106611885
STRU115205380
OTW112220780 112255654
OMY110488634 110504839
OGO123993270 124010849
ONE115129348 115130544
OKI109864656 109905077
OKE118363382 118384016
SALP112069063
SNH120047075 120062128
CCLU121579852
ELS105025204(carns1)
SFM108935907(carns1)
AANG118231339
LOC102683811 102697335(carns1)
PSPA121307257 121307276
ARUT117409973 117410067 117410068 117966719 117966722 117968602 117970752
PSEX120538030
PMRN116936845(CARNS1)
PCAN112554628
CRG105317679 105324334 105346080
CANU128155782 128191493 128193255
CVN111125295 111125850
OED125667616 125667619 125673609
MYI110448302
PMAX117317717
MCAF127717988
MMER123525736 123534519 123541963 123542593
RPHI132745599 132755221
DPOL127831335 127831459 127835619 127835874 127835878 127840179 127842007 127846254 127847009 127847010 127847019 127848476
MAEA128209787 128227124 128227126 128241452
OBI106877889 106879380
OSN115220804 115220869 115220883 115220906
LJP135477681 135478135 135478138
LAK106157795
 » show all
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 234:3210-8 (1959)
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 77:575-81 (1960)
DOI:10.1042/bj0770575
Reference
3  [PMID:4988625]
  Authors
Crush KG
  Title
Carnosine and related substances in animal tissues.
  Journal
Comp Biochem Physiol 34:3-30 (1970)
DOI:10.1016/0010-406x(70)90049-6
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 285:9346-56 (2010)
DOI:10.1074/jbc.M109.095505
  Sequence
[gga:100359387] [hsa:57571] [mmu:107239]
Other DBs
ExplorEnz - The Enzyme Database: 6.3.2.11
IUBMB Enzyme Nomenclature: 6.3.2.11
ExPASy - ENZYME nomenclature database: 6.3.2.11
BRENDA, the Enzyme Database: 6.3.2.11
CAS: 9023-61-4
LinkDB

KEGG   REACTION: R01991
Entry
R01991                      Reaction                               
Name
L-histidine:4-aminobutanoate ligase (ADP-forming)
Definition
ATP + L-Histidine + 4-Aminobutanoate <=> ADP + Orthophosphate + Homocarnosine
Equation
Reaction class
RC00002  C00002_C00008
RC00064  C00135_C00884
RC00090  C00334_C00884
Enzyme
Pathway
rn00330  Arginine and proline metabolism
rn01100  Metabolic pathways
Brite
Enzymatic reactions [BR:br08201]
 6. Ligase reactions
  6.3  Forming carbon-nitrogen bonds
   6.3.2  Acid-D-amino-acid ligases (peptide synthases)
    6.3.2.11
     R01991  ATP + L-Histidine + 4-Aminobutanoate <=> ADP + Orthophosphate + Homocarnosine
Orthology
K14755  carnosine synthase [EC:6.3.2.11]
Other DBs
RHEA: 59571
LinkDB

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