KEGG   ORTHOLOGY: K01948
Entry
K01948                      KO                                     
Symbol
CPS1
Name
carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
Pathway
map00220  Arginine biosynthesis
map00250  Alanine, aspartate and glutamate metabolism
map00910  Nitrogen metabolism
map01100  Metabolic pathways
map01120  Microbial metabolism in diverse environments
map01200  Carbon metabolism
map01230  Biosynthesis of amino acids
Module
M00029  Urea cycle
Reaction
R00149  carbon-dioxide:ammonia ligase (ADP-forming,carbamate-phosphorylating)
Disease
H00164  Carbamoyl phosphate synthetase I deficiency
H01398  Primary hyperammonemia (Urea cycle disorders)
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09102 Energy metabolism
   00910 Nitrogen metabolism
    K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
  09105 Amino acid metabolism
   00250 Alanine, aspartate and glutamate metabolism
    K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
   00220 Arginine biosynthesis
    K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors
    K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
Enzymes [BR:ko01000]
 6. Ligases
  6.3  Forming carbon-nitrogen bonds
   6.3.4  Other carbon-nitrogen ligases
    6.3.4.16  carbamoyl-phosphate synthase (ammonia)
     K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
Peptidases and inhibitors [BR:ko01002]
 Cysteine peptidases
  Family C26: gamma-glutamyl hydrolase family
   K01948  CPS1; carbamoyl-phosphate synthase (ammonia)
Other DBs
GO: 0004087
Genes
HSA: 1373(CPS1)
PTR: 738670(CPS1)
PPS: 100984596(CPS1)
GGO: 101130709(CPS1)
PON: 100442727(CPS1)
PPYG: 129031297(CPS1)
NLE: 100596216(CPS1)
HMH: 116810358(CPS1)
SSYN: 129488372(CPS1)
MCC: 711806(CPS1)
MCF: 102139369(CPS1)
MTHB: 126932526
MNI: 105481139(CPS1)
CSAB: 103217763(CPS1)
CATY: 105580142(CPS1)
PANU: 100998090(CPS1)
TGE: 112636758(CPS1)
MLEU: 105529410(CPS1)
RRO: 104655495(CPS1)
RBB: 108540334(CPS1)
TFN: 117094576(CPS1)
PTEH: 111548015(CPS1)
CANG: 105504766(CPS1)
CJC: 100402602(CPS1)
SBQ: 101050943(CPS1)
CIMI: 108316738(CPS1)
ANAN: 105712873(CPS1)
CSYR: 103265850(CPS1)
MMUR: 105863911(CPS1)
LCAT: 123643910(CPS1)
PCOQ: 105823919(CPS1)
OGA: 100951321(CPS1)
MMU: 227231(Cps1)
MCAL: 110289777(Cps1)
MPAH: 110322614(Cps1)
RNO: 497840(Cps1)
MCOC: 116089210(Cps1)
ANU: 117705509(Cps1)
ASYL: 127692324(Cps1)
MUN: 110539643(Cps1)
CGE: 100760467(Cps1)
MAUA: 101832270(Cps1)
PROB: 127226400(Cps1)
PLEU: 114693613(Cps1)
MORG: 121433434(Cps1)
MFOT: 126508819
AAMP: 119820686(Cps1)
NGI: 103744467(Cps1)
HGL: 101696508(Cps1)
CPOC: 100723563(Cps1)
CCAN: 109699651(Cps1)
DORD: 105995361(Cps1)
DSP: 122107420(Cps1)
PLOP: 125349917(Cps1)
NCAR: 124979792
MMMA: 107138386(Cps1)
ITI: 101969408(Cps1)
OCU: 100303765
OPI: 101527604(CPS1)
TUP: 102491899(CPS1)
GVR: 103596711(CPS1)
CFA: 488506(CPS1)
CLUD: 112656390(CPS1)
VVP: 112922588(CPS1)
VLG: 121480750(CPS1)
NPO: 129522784(CPS1)
AML: 100466842(CPS1)
UMR: 103659822(CPS1)
UAH: 113250504(CPS1)
UAR: 123798268(CPS1)
ELK: 111156350
LLV: 125095184
MPUF: 101674281(CPS1)
MNP: 132013515(CPS1)
MLK: 131827499(CPS1)
NVS: 122902267(CPS1)
ORO: 101371212(CPS1)
EJU: 114215934(CPS1)
ZCA: 113920213(CPS1)
MLX: 118012083(CPS1)
NSU: 110591845(CPS1)
LWW: 102728411(CPS1)
FCA: 101099824(CPS1)
PYU: 121031013(CPS1)
PCOO: 112866839(CPS1)
PBG: 122481726(CPS1)
PVIV: 125172285(CPS1)
LRUF: 124515815
PTG: 102968283(CPS1)
PPAD: 109265124(CPS1)
PUC: 125911234
AJU: 106984775
HHV: 120243755(CPS1)
BTA: 510506(CPS1)
BOM: 102267831(CPS1)
BIU: 109570387(CPS1)
BBUB: 102415970(CPS1)
BBIS: 105003728(CPS1)
CHX: 102179612(CPS1)
OAS: 101107584(CPS1)
BTAX: 128067321(CPS1)
CSUM: 138075664(CPS1)
ODA: 120854532(CPS1)
CCAD: 122426621(CPS1)
MREE: 136164948(CPS1)
OVR: 110135844(CPS1)
MBEZ: 129538560(CPS1)
SSC: 100157716(CPS1)
CFR: 102516394(CPS1)
CBAI: 105072943(CPS1)
CDK: 105101586(CPS1)
VPC: 102537219(CPS1)
BACU: 103003987(CPS1)
BMUS: 118898574(CPS1)
LVE: 103079188(CPS1)
OOR: 101279675(CPS1)
DLE: 111171789(CPS1)
PCAD: 102973240(CPS1)
PSIU: 116756616(CPS1)
NASI: 112411855(CPS1)
ECB: 100050116(CPS1)
EPZ: 103554808(CPS1)
EAI: 106843053(CPS1)
MYB: 102262103(CPS1)
MYD: 102756563(CPS1)
MMYO: 118661701(CPS1)
MLF: 102436612(CPS1)
MDT: 132238193(CPS1)
PKL: 118709020(CPS1)
EFUS: 103289343(CPS1)
MNA: 107524839(CPS1)
DRO: 112307671(CPS1)
SHON: 118984231(CPS1)
AJM: 119044341(CPS1)
PDIC: 114494376(CPS1)
PHAS: 123828195(CPS1)
MMF: 118625808(CPS1)
PPAM: 129080372(CPS1)
HAI: 109390430(CPS1)
RFQ: 117025658(CPS1)
PALE: 102895317(CPS1)
PGIG: 120587605(CPS1)
PVP: 105291095(CPS1)
RAY: 107517643(CPS1)
MJV: 108396990(CPS1)
TOD: 119256167(CPS1)
SARA: 101556847(CPS1)
SFUM: 130021094(CPS1)
SETR: 126023186(CPS1)
LAV: 100669826(CPS1)
TMU: 101359193
ETF: 101651869(CPS1)
DNM: 101436387(CPS1)
MDO: 100015090(CPS1)
GAS: 123242723(CPS1)
SHR: 100929825(CPS1)
AFZ: 127553150
PCW: 110223299(CPS1)
TVP: 118847442(CPS1)
PBRV: 138151580(CPS1)
OAA: 100077228(CPS1)
TACU: 119930485(CPS1)
GGA: 428994(CPS1)
PCOC: 116235914(CPS1)
MGP: 100539661(CPS1)
CJO: 107316483(CPS1)
TPAI: 128076845(CPS1)
LMUT: 125697109(CPS1)
NMEL: 110399288(CPS1)
APLA: 101797791(CPS1)
ACYG: 106034508(CPS1)
CATA: 118260689(CPS1)
AFUL: 116490822(CPS1)
TGU: 100221389(CPS1)
LSR: 110471694(CPS1)
SCAN: 103814751(CPS1)
PMOA: 120511507(CPS1)
OTC: 121339687(CPS1)
PRUF: 121364445(CPS1)
GFR: 102044867(CPS1)
FAB: 101816308(CPS1)
OMA: 130255623(CPS1)
PHI: 102105821(CPS1)
PMAJ: 107207199(CPS1)
CCAE: 111931881(CPS1)
CCW: 104694540(CPS1)
CBRC: 103624329(CPS1)
ACOE: 138113499(CPS1)
ETL: 114059416(CPS1)
ZAB: 102066900(CPS1)
ZLE: 135450165(CPS1)
ACHL: 103807118(CPS1)
SVG: 106856551(CPS1)
MMEA: 130588855(CPS1)
HRT: 120754985(CPS1)
SATI: 136363311(CPS1)
FPG: 101910440(CPS1)
FCH: 102046837(CPS1)
CCRI: 104161143(CPS1)
NNT: 104405431(CPS1)
SHAB: 115608976(CPS1)
ACUN: 113482015(CPS1)
TALA: 104368683(CPS1)
ACHC: 115343371(CPS1)
HALD: 104321187
HLE: 104841537(CPS1)
AGEN: 126039478
HHAR: 128144475(CPS1)
GCL: 127018302
LDI: 104350520(CPS1)
MNB: 103771914(CPS1)
DPUB: 104299635
PPUS: 135186284(CPS1)
AVIT: 104278583(CPS1)
BRHI: 104497824(CPS1)
EGZ: 104124302(CPS1)
NNI: 104009921(CPS1)
PCRI: 104030248(CPS1)
PCAO: 104045913(CPS1)
PADL: 103921645(CPS1)
AFOR: 103900404(CPS1)
FGA: 104075389(CPS1)
GSTE: 104254124(CPS1)
CLV: 102091543(CPS1)
MUI: 104538785(CPS1)
PGUU: 104466714(CPS1)
PLET: 104627545(CPS1)
EHS: 104510972
CMAC: 104477513(CPS1)
CUCA: 104064581(CPS1)
TEO: 104379491(CPS1)
BREG: 104643520(CPS1)
OHA: 104338581(CPS1)
ACAR: 104531757(CPS1)
CPEA: 104388101(CPS1)
CVF: 104288744(CPS1)
RTD: 128912324(CPS1)
AAM: 106492382(CPS1)
AROW: 112977009(CPS1)
NPD: 112955714(CPS1)
TGT: 104578213(CPS1)
DNE: 112996164(CPS1)
SCAM: 104153019(CPS1)
ASN: 102386005(CPS1)
AMJ: 106736782(CPS1)
CPOO: 109323109(CPS1)
GGN: 109301987(CPS1)
PSS: 102462440(CPS1)
CMY: 102943091(CPS1)
CCAY: 125645099(CPS1)
DCC: 119863334(CPS1)
CPIC: 101942404(CPS1)
TST: 117884622(CPS1)
CABI: 116816082(CPS1)
MRV: 120374564(CPS1)
ACS: 100562333(cps1)
ASAO: 132778352(CPS1)
PVT: 110072386(CPS1)
SUND: 121919956(CPS1)
PBI: 103061484(CPS1)
PMUR: 107301875(CPS1)
CTIG: 120312340(CPS1)
TSR: 106557156(CPS1)
PGUT: 117674372(CPS1)
PTEX: 113440996(CPS1)
NSS: 113415638(CPS1)
VKO: 123024301(CPS1)
PMUA: 114583459(CPS1)
PRAF: 128401532(CPS1)
ZVI: 118096710(CPS1)
HCG: 128339456(CPS1) 128340704
EMC: 129324191(CPS1)
XLA: 733340(cps1.L)
XTR: 100158506(cps1)
NPR: 108794421(CPS1)
RTEM: 120943863(CPS1)
BBUF: 121007628(CPS1)
BGAR: 122945871(CPS1)
MUO: 115473875(CPS1)
GSH: 117361602(CPS1)
DRE: 555623(cps1)
SRX: 107741925
SANH: 107660611
SGH: 107555908(cps1)
CAUA: 113108375(cps1)
CGIB: 127964800
PTET: 122351718(cps1)
LROH: 127170387(cps1)
OMC: 131547203(cps1)
PPRM: 120461485 120461522(cps1)
RKG: 130096638(cps1)
MAMB: 125270576(cps1)
CIDE: 127519252
CERY: 137035481(cps1)
MASI: 127447239
IPU: 108266807
IFU: 128608489(cps1)
PHYP: 113526109(cps1)
SMEO: 124383048(cps1)
TFD: 113648186(cps1)
TVC: 132850489(cps1)
AMEX: 103026142(cps1)
CMAO: 118812551(cps1)
EEE: 113589154(cps1)
CHAR: 105908013(cps1)
SPIL: 134078118(cps1)
TRU: 101074466(cps1)
TFS: 130529796(cps1)
LCO: 104929821(cps1)
NCC: 104953308(cps1)
TBEN: 117485740(cps1)
CGOB: 115026190(cps1)
PGEO: 117466178(cps1)
GACU: 117538394(cps1)
EMAC: 134867667(cps1)
ELY: 117251123(cps1)
EFO: 125899448(cps1)
PLEP: 121949412(cps1)
SLUC: 116037726(cps1) 116050235
ECRA: 117952874(cps1)
ESP: 116698408(cps1)
PFLV: 114563820(cps1)
GAT: 120834388(cps1)
PPUG: 119229014(cps1)
AFB: 129104485(cps1)
CLUM: 117750160(cps1)
PSWI: 130200187(cps1)
CANA: 137782647(cps1)
CVE: 137866664(cps1)
MSAM: 119903960(cps1)
SCHU: 122885023(cps1)
CUD: 121523069(cps1)
SPUL: 138592082(cps1)
LMIX: 132987268(cps1)
ALAT: 119025840(cps1)
ASTR: 137604783(cps1)
MZE: 101479159(cps1)
ONL: 100696002(cps1)
OAU: 116312077(cps1)
OLA: 101164854(cps1)
OML: 112155538(cps1)
CSAI: 133446042(cps1)
XMA: 102232024(cps1)
XCO: 114147479(cps1)
XHE: 116723745(cps1)
PRET: 103461545(cps1)
PFOR: 103141450(cps1)
PLAI: 106948981(cps1)
PMEI: 106918783(cps1)
GAF: 122839359(cps1)
PPRL: 129361497(cps1)
CVG: 107088030(cps1)
CTUL: 119798621(cps1)
GMU: 124871823(cps1)
NFU: 107396242(cps1)
KMR: 108240446(cps1)
ALIM: 106519746(cps1)
NWH: 119418844(cps1)
AOCE: 111584991(cps1)
EJA: 138201249(cps1)
BFRE: 138620898(cps1)
MCEP: 125018029(cps1)
CSEM: 103392237(cps1)
POV: 109631134(cps1)
SSEN: 122765088(cps1)
HHIP: 117755012(cps1)
HSP: 118119733(cps1)
PPLT: 128456322(cps1)
SMAU: 118282879(cps1)
LCF: 108892757
SDU: 111218322(cps1)
SLAL: 111662716(cps1)
XGL: 120801664(cps1)
HCQ: 109531202(cps1)
SBIA: 133512413(cps1)
PEE: 133410390(cps1)
PTAO: 133486970(cps1)
BPEC: 110156044(cps1)
BSPL: 114847503(cps1)
SJO: 128368364(cps1)
SSCO: 133992618(cps1)
SASA: 106586447(cps1)
STRU: 115160979(cps1)
OTW: 112238893(cps1)
OMY: 100136758(cps1)
OGO: 124044011(cps1)
ONE: 115111283(cps1)
OKI: 109868274(cps1)
OKE: 118366886(cps1)
OMM: 135519281(cps1)
SALP: 112068953(cps1)
SNH: 120058044(cps1)
CCLU: 121536995(cps1)
ELS: 105008497(cps1)
SFM: 108922278(cps1)
AANG: 118224050(cps1)
AROT: 135251771(cps1)
CCOG: 133123781(cps1)
LOC: 102687173(cps1)
PSPA: 121323136(cps1)
ARUT: 117412900(cps1)
PSEX: 120530771(cps1)
LCM: 102363877(CPS1)
CMK: 103182939(cps1)
RTP: 109937803(cps1)
CPLA: 122551475(cps1)
HOC: 132817290(cps1)
LERI: 129698851(cps1)
BFO: 118405554
BBEL: 109465412
SPU: 584369
LPIC: 129257812
APLC: 110989285
ARUN: 117291237
AJC: 117122950
SKO: 100367850
PFLL: 139143450
PCAN: 112558598
LSAX: 138959938
BGT: 106078424
GAE: 121387415
HRF: 124119867
HRJ: 124262534
HASI: 137298909
OBI: 106879763
OSN: 115215632
LJP: 135464549
EPA: 110246605
ATEN: 116288266
AMIL: 114968085
MCAZ: 138049324
MFOO: 137985546
PDAM: 113673921
SPIS: 111323363
DGT: 114517513
XEN: 124452820
RES: 135681610
FCY: FRACYDRAFT_169332(Fc_169332)
AAF: AURANDRAFT_20552(CPS1)
BLAC: 94352586(CCR75_008868)
SPAR: SPRG_05438
 » show all
Reference
PMID:1840546
  Authors
Haraguchi Y, Uchino T, Takiguchi M, Endo F, Mori M, Matsuda I
  Title
Cloning and sequence of a cDNA encoding human carbamyl phosphate synthetase I: molecular analysis of hyperammonemia.
  Journal
Gene 107:335-40 (1991)
DOI:10.1016/0378-1119(91)90336-A
  Sequence
[hsa:1373]
LinkDB

KEGG   ENZYME: 6.3.4.16
Entry
EC 6.3.4.16                 Enzyme                                 
Name
carbamoyl-phosphate synthase (ammonia);
carbon-dioxide---ammonia ligase;
carbamoylphosphate synthase;
carbamylphosphate synthetase;
carbamoylphosphate synthase (ammonia);
carbamoylphosphate synthetase;
carbamylphosphate synthetase I;
CPSI (gene name);
carbon-dioxide:ammonia ligase (ADP-forming, carbamate-phosphorylating)
Class
Ligases;
Forming carbon-nitrogen bonds;
Other carbon-nitrogen ligases
Sysname
hydrogencarbonate:ammonia ligase (ADP-forming, carbamate-phosphorylating)
Reaction(IUBMB)
2 ATP + NH3 + hydrogencarbonate = 2 ADP + phosphate + carbamoyl phosphate (overall reaction) [RN:R00149];
(1a) ATP + hydrogencarbonate = ADP + carboxyphosphate;
(1b) NH3 + carboxyphosphate = carbamate + phosphate;
(1c) ATP + carbamate = ADP + carbamoyl phosphate
Reaction(KEGG)
R00149
Substrate
ATP [CPD:C00002];
NH3 [CPD:C00014];
hydrogencarbonate [CPD:C00288];
carboxyphosphate [CPD:C20969];
carbamate [CPD:C01563]
Product
ADP [CPD:C00008];
phosphate [CPD:C00009];
carbamoyl phosphate [CPD:C00169];
carboxyphosphate [CPD:C20969];
carbamate [CPD:C01563]
Comment
The enzyme catalyses the first committed step in the urea cycle. The reaction proceeds via three separate chemical reactions: phosphorylation of hydrogencarbonate to carboxyphosphate; a nucleophilic attack of ammonia on carboxyphosphate yielding carbamate; and the phosphorylation of carbamate forming carbamoyl phosphate. Two moles of ATP are utilized for the synthesis of one molecule of carbamyl phosphate, making the reaction essentially irreversible. The enzyme requires the allosteric activator N-acetyl-L-glutamate. cf. EC 6.3.5.5, carbamoyl-phosphate synthase (glutamine-hydrolysing).
History
EC 6.3.4.16 created 1965 as EC 2.7.2.5, transferred 1978 to EC 6.3.4.16
Pathway
ec00220  Arginine biosynthesis
ec00250  Alanine, aspartate and glutamate metabolism
ec00910  Nitrogen metabolism
ec01100  Metabolic pathways
ec01120  Microbial metabolism in diverse environments
Orthology
K01948  carbamoyl-phosphate synthase (ammonia)
Genes
HSA1373(CPS1)
PTR738670(CPS1)
PPS100984596(CPS1)
GGO101130709(CPS1)
PON100442727(CPS1)
PPYG129031297(CPS1)
NLE100596216(CPS1)
HMH116810358(CPS1)
SSYN129488372(CPS1)
MCC711806(CPS1)
MCF102139369(CPS1)
MTHB126932526
MNI105481139(CPS1)
CSAB103217763(CPS1)
CATY105580142(CPS1)
PANU100998090(CPS1)
TGE112636758(CPS1)
MLEU105529410(CPS1)
RRO104655495(CPS1)
RBB108540334(CPS1)
TFN117094576(CPS1)
PTEH111548015(CPS1)
CANG105504766(CPS1)
CJC100402602(CPS1)
SBQ101050943(CPS1)
CIMI108316738(CPS1)
ANAN105712873(CPS1)
CSYR103265850(CPS1)
MMUR105863911(CPS1)
LCAT123643910(CPS1)
PCOQ105823919(CPS1)
OGA100951321(CPS1)
MMU227231(Cps1)
MCAL110289777(Cps1)
MPAH110322614(Cps1)
RNO497840(Cps1)
MCOC116089210(Cps1)
ANU117705509(Cps1)
ASYL127692324(Cps1)
MUN110539643(Cps1)
CGE100760467(Cps1)
MAUA101832270(Cps1)
PROB127226400(Cps1)
PLEU114693613(Cps1)
MORG121433434(Cps1)
MFOT126508819
AAMP119820686(Cps1)
NGI103744467(Cps1)
HGL101696508(Cps1)
CPOC100723563(Cps1)
CCAN109699651(Cps1)
DORD105995361(Cps1)
DSP122107420(Cps1)
PLOP125349917(Cps1)
NCAR124979792
MMMA107138386(Cps1)
ITI101969408(Cps1)
OCU100303765
OPI101527604(CPS1)
TUP102491899(CPS1)
GVR103596711(CPS1)
CFA488506(CPS1)
CLUD112656390(CPS1)
VVP112922588(CPS1)
VLG121480750(CPS1)
NPO129522784(CPS1)
AML100466842(CPS1)
UMR103659822(CPS1)
UAH113250504(CPS1)
UAR123798268(CPS1)
ELK111156350
LLV125095184
MPUF101674281(CPS1)
MNP132013515(CPS1)
MLK131827499(CPS1)
NVS122902267(CPS1)
ORO101371212(CPS1)
EJU114215934(CPS1)
ZCA113920213(CPS1)
MLX118012083(CPS1)
NSU110591845(CPS1)
LWW102728411(CPS1)
FCA101099824(CPS1)
PYU121031013(CPS1)
PCOO112866839(CPS1)
PBG122481726(CPS1)
PVIV125172285(CPS1)
LRUF124515815
PTG102968283(CPS1)
PPAD109265124(CPS1)
PUC125911234
AJU106984775
HHV120243755(CPS1)
BTA510506(CPS1)
BOM102267831(CPS1)
BIU109570387(CPS1)
BBUB102415970(CPS1)
BBIS105003728(CPS1)
CHX102179612(CPS1)
OAS101107584(CPS1)
BTAX128067321(CPS1)
CSUM138075664(CPS1)
ODA120854532(CPS1)
CCAD122426621(CPS1)
MREE136164948(CPS1)
OVR110135844(CPS1)
MBEZ129538560(CPS1)
SSC100157716(CPS1)
CFR102516394(CPS1)
CBAI105072943(CPS1)
CDK105101586(CPS1)
VPC102537219(CPS1)
BACU103003987(CPS1)
BMUS118898574(CPS1)
LVE103079188(CPS1)
OOR101279675(CPS1)
DLE111171789(CPS1)
PCAD102973240(CPS1)
PSIU116756616(CPS1)
NASI112411855(CPS1)
ECB100050116(CPS1)
EPZ103554808(CPS1)
EAI106843053(CPS1)
MYB102262103(CPS1)
MYD102756563(CPS1)
MMYO118661701(CPS1)
MLF102436612(CPS1)
MDT132238193(CPS1)
PKL118709020(CPS1)
EFUS103289343(CPS1)
MNA107524839(CPS1)
DRO112307671(CPS1)
SHON118984231(CPS1)
AJM119044341(CPS1)
PDIC114494376(CPS1)
PHAS123828195(CPS1)
MMF118625808(CPS1)
PPAM129080372(CPS1)
HAI109390430(CPS1)
RFQ117025658(CPS1)
PALE102895317(CPS1)
PGIG120587605(CPS1)
PVP105291095(CPS1)
RAY107517643(CPS1)
MJV108396990(CPS1)
TOD119256167(CPS1)
SARA101556847(CPS1)
SFUM130021094(CPS1)
SETR126023186(CPS1)
LAV100669826(CPS1)
TMU101359193
ETF101651869(CPS1)
DNM101436387(CPS1)
MDO100015090(CPS1)
GAS123242723(CPS1)
SHR100929825(CPS1)
AFZ127553150
PCW110223299(CPS1)
TVP118847442(CPS1)
PBRV138151580(CPS1)
OAA100077228(CPS1)
TACU119930485(CPS1)
GGA428994(CPS1)
PCOC116235914(CPS1)
MGP100539661(CPS1)
CJO107316483(CPS1)
TPAI128076845(CPS1)
LMUT125697109(CPS1)
NMEL110399288(CPS1)
APLA101797791(CPS1)
ACYG106034508(CPS1)
CATA118260689(CPS1)
AFUL116490822(CPS1)
TGU100221389(CPS1)
LSR110471694(CPS1)
SCAN103814751(CPS1)
PMOA120511507(CPS1)
OTC121339687(CPS1)
PRUF121364445(CPS1)
GFR102044867(CPS1)
FAB101816308(CPS1)
OMA130255623(CPS1)
PHI102105821(CPS1)
PMAJ107207199(CPS1)
CCAE111931881(CPS1)
CCW104694540(CPS1)
CBRC103624329(CPS1)
ACOE138113499(CPS1)
ETL114059416(CPS1)
ZAB102066900(CPS1)
ZLE135450165(CPS1)
ACHL103807118(CPS1)
SVG106856551(CPS1)
MMEA130588855(CPS1)
HRT120754985(CPS1)
SATI136363311(CPS1)
FPG101910440(CPS1)
FCH102046837(CPS1)
CCRI104161143(CPS1)
NNT104405431(CPS1)
SHAB115608976(CPS1)
ACUN113482015(CPS1)
TALA104368683(CPS1)
ACHC115343371(CPS1)
HALD104321187
HLE104841537(CPS1)
AGEN126039478
HHAR128144475(CPS1)
GCL127018302
LDI104350520(CPS1)
MNB103771914(CPS1)
DPUB104299635
PPUS135186284(CPS1)
AVIT104278583(CPS1)
BRHI104497824(CPS1)
EGZ104124302(CPS1)
NNI104009921(CPS1)
PCRI104030248(CPS1)
PCAO104045913(CPS1)
PADL103921645(CPS1)
AFOR103900404(CPS1)
FGA104075389(CPS1)
GSTE104254124(CPS1)
CLV102091543(CPS1)
MUI104538785(CPS1)
PGUU104466714(CPS1)
PLET104627545(CPS1)
EHS104510972
CMAC104477513(CPS1)
CUCA104064581(CPS1)
TEO104379491(CPS1)
BREG104643520(CPS1)
OHA104338581(CPS1)
ACAR104531757(CPS1)
CPEA104388101(CPS1)
CVF104288744(CPS1)
RTD128912324(CPS1)
AAM106492382(CPS1)
AROW112977009(CPS1)
NPD112955714(CPS1)
TGT104578213(CPS1)
DNE112996164(CPS1)
SCAM104153019(CPS1)
ASN102386005(CPS1)
AMJ106736782(CPS1)
CPOO109323109(CPS1)
GGN109301987(CPS1)
PSS102462440(CPS1)
CMY102943091(CPS1)
CCAY125645099(CPS1)
DCC119863334(CPS1)
CPIC101942404(CPS1)
TST117884622(CPS1)
CABI116816082(CPS1)
MRV120374564(CPS1)
ACS100562333(cps1)
ASAO132778352(CPS1)
PVT110072386(CPS1)
SUND121919956(CPS1)
PBI103061484(CPS1)
PMUR107301875(CPS1)
CTIG120312340(CPS1)
TSR106557156(CPS1)
PGUT117674372(CPS1)
PTEX113440996(CPS1)
NSS113415638(CPS1)
VKO123024301(CPS1)
PMUA114583459(CPS1)
PRAF128401532(CPS1)
ZVI118096710(CPS1)
HCG128339456(CPS1) 128340704
EMC129324191(CPS1)
XLA733340(cps1.L)
XTR100158506(cps1)
NPR108794421(CPS1)
RTEM120943863(CPS1)
BBUF121007628(CPS1)
BGAR122945871(CPS1)
MUO115473875(CPS1)
GSH117361602(CPS1)
DRE555623(cps1)
SRX107741925
SANH107660611
SGH107555908(cps1)
CAUA113108375(cps1)
CGIB127964800
PTET122351718(cps1)
LROH127170387(cps1)
OMC131547203(cps1)
PPRM120461485 120461522(cps1)
RKG130096638(cps1)
MAMB125270576(cps1)
CIDE127519252
CERY137035481(cps1)
MASI127447239
IPU108266807
IFU128608489(cps1)
PHYP113526109(cps1)
SMEO124383048(cps1)
TFD113648186(cps1)
TVC132850489(cps1)
AMEX103026142(cps1)
CMAO118812551(cps1)
EEE113589154(cps1)
CHAR105908013(cps1)
SPIL134078118(cps1)
TRU101074466(cps1)
TFS130529796(cps1)
TNGGSTEN00004415G001
LCO104929821(cps1)
NCC104953308(cps1)
TBEN117485740(cps1)
CGOB115026190(cps1)
PGEO117466178(cps1)
GACU117538394(cps1)
EMAC134867667(cps1)
ELY117251123(cps1)
EFO125899448(cps1)
PLEP121949412(cps1)
SLUC116037726(cps1) 116050235
ECRA117952874(cps1)
ESP116698408(cps1)
PFLV114563820(cps1)
GAT120834388(cps1)
PPUG119229014(cps1)
AFB129104485(cps1)
CLUM117750160(cps1)
PSWI130200187(cps1)
CANA137782647(cps1)
CVE137866664(cps1)
MSAM119903960(cps1)
SCHU122885023(cps1)
CUD121523069(cps1)
SPUL138592082(cps1)
LMIX132987268(cps1)
ALAT119025840(cps1)
ASTR137604783(cps1)
MZE101479159(cps1)
ONL100696002(cps1)
OAU116312077(cps1)
OLA101164854(cps1)
OML112155538(cps1)
CSAI133446042(cps1)
XMA102232024(cps1)
XCO114147479(cps1)
XHE116723745(cps1)
PRET103461545(cps1)
PFOR103141450(cps1)
PLAI106948981(cps1)
PMEI106918783(cps1)
GAF122839359(cps1)
PPRL129361497(cps1)
CVG107088030(cps1)
CTUL119798621(cps1)
GMU124871823(cps1)
NFU107396242(cps1)
KMR108240446(cps1)
ALIM106519746(cps1)
NWH119418844(cps1)
AOCE111584991(cps1)
EJA138201249(cps1)
BFRE138620898(cps1)
MCEP125018029(cps1)
CSEM103392237(cps1)
POV109631134(cps1)
SSEN122765088(cps1)
HHIP117755012(cps1)
HSP118119733(cps1)
PPLT128456322(cps1)
SMAU118282879(cps1)
LCF108892757
SDU111218322(cps1)
SLAL111662716(cps1)
XGL120801664(cps1)
HCQ109531202(cps1)
SBIA133512413(cps1)
PEE133410390(cps1)
PTAO133486970(cps1)
BPEC110156044(cps1)
BSPL114847503(cps1)
SJO128368364(cps1)
SSCO133992618(cps1)
SASA106586447(cps1)
STRU115160979(cps1)
OTW112238893(cps1)
OMY100136758(cps1)
OGO124044011(cps1)
ONE115111283(cps1)
OKI109868274(cps1)
OKE118366886(cps1)
OMM135519281(cps1)
SALP112068953(cps1)
SNH120058044(cps1)
CCLU121536995(cps1)
ELS105008497(cps1)
SFM108922278(cps1)
AANG118224050(cps1)
AROT135251771(cps1)
CCOG133123781(cps1)
LOC102687173(cps1)
PSPA121323136(cps1)
ARUT117412900(cps1)
PSEX120530771(cps1)
LCM102363877(CPS1)
CMK103182939(cps1)
RTP109937803(cps1)
CPLA122551475(cps1)
HOC132817290(cps1)
LERI129698851(cps1)
BFO118405554
BBEL109465412
SCLV120327643 120328367
SPU584369
LPIC129257812
APLC110989285
ARUN117291237
AJC117122950
SKO100367850
PFLL139143450
LGILOTGIDRAFT_137874
PCAN112558598
LSAX138959938
BGT106078424
GAE121387415
HRF124119867
HRJ124262534
HASI137298909
OBI106879763
OSN115215632
LJP135464549
LAK106172324 106173741
NVE5504557 5515702
EPA110246605
ATEN116288266
ADF107350432 107350434
AMIL114968085
MCAZ138049324
MFOO137985546
PDAM113673921
SPIS111323363
DGT114517513
XEN124452820
RES135681610
PTIPHATRDRAFT_24195
FCYFRACYDRAFT_169332(Fc_169332)
TPSTHAPSDRAFT_40323
AAFAURANDRAFT_20552(CPS1)
PIFPITG_03098
PSOJPHYSODRAFT_348996
BLAC94352586(CCR75_008868)
SPARSPRG_05438
 » show all
Reference
1  [PMID:14213379]
  Authors
FAHIEN LA, COHEN PP.
  Title
A KINETIC STUDY OF CARBAMYL PHOSPHATE SYNTHETASE.
  Journal
J Biol Chem 239:1925-34 (1964)
Reference
2  [PMID:13790558]
  Authors
JONES ME, SPECTOR L.
  Title
The pathway of carbonate in the biosynthesis of carbamyl phosphate.
  Journal
J Biol Chem 235:2897-901 (1960)
Reference
3  [PMID:13563449]
  Authors
METZENBERG RL, MARSHALL M, COHEN PP.
  Title
Purification of carbamyl phosphate synthetase from frog liver.
  Journal
J Biol Chem 233:102-5 (1958)
Reference
4  [PMID:26151989]
  Authors
Marshall M, Metzenberg RL, Cohen PP
  Title
Physical and Kinetic Properties of Carbamyl Phosphate Synthetase from Frog Liver.
  Journal
J Biol Chem 236:2229-37 (1961)
Reference
5  [PMID:6249820]
  Authors
Pierson DL, Brien JM
  Title
Human carbamylphosphate synthetase I. Stabilization, purification, and partial characterization of the enzyme from human liver.
  Journal
J Biol Chem 255:7891-5 (1980)
Reference
6  [PMID:19754428]
  Authors
Pekkala S, Martinez AI, Barcelona B, Gallego J, Bendala E, Yefimenko I, Rubio V, Cervera J
  Title
Structural insight on the control of urea synthesis: identification of the binding site for N-acetyl-L-glutamate, the essential allosteric activator of mitochondrial carbamoyl phosphate synthetase.
  Journal
Biochem J 424:211-20 (2009)
DOI:10.1042/BJ20090888
  Sequence
[hsa:1373]
Other DBs
ExplorEnz - The Enzyme Database: 6.3.4.16
IUBMB Enzyme Nomenclature: 6.3.4.16
ExPASy - ENZYME nomenclature database: 6.3.4.16
BRENDA, the Enzyme Database: 6.3.4.16
CAS: 9026-23-7
LinkDB

KEGG   REACTION: R00149
Entry
R00149                      Reaction                               
Name
carbon-dioxide:ammonia ligase (ADP-forming,carbamate-phosphorylating)
Definition
2 ATP + Ammonia + CO2 + H2O <=> 2 ADP + Orthophosphate + Carbamoyl phosphate
Equation
Reaction class
RC00002  C00002_C00008
RC02804  C00014_C00169
Enzyme
Pathway
rn00220  Arginine biosynthesis
rn00250  Alanine, aspartate and glutamate metabolism
rn00910  Nitrogen metabolism
rn01100  Metabolic pathways
rn01120  Microbial metabolism in diverse environments
rn01200  Carbon metabolism
rn01230  Biosynthesis of amino acids
Module
M00029  Urea cycle
Brite
Enzymatic reactions [BR:br08201]
 6. Ligase reactions
  6.3  Forming carbon-nitrogen bonds
   6.3.4  Other carbon-nitrogen ligases
    6.3.4.16
     R00149  2 ATP + Ammonia + CO2 + H2O <=> 2 ADP + Orthophosphate + Carbamoyl phosphate
Orthology
K01948  carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
LinkDB

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