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)
NLE100596216(CPS1)
MCC711806(CPS1)
MCF102139369(CPS1)
CSAB103217763(CPS1)
CATY105580142(CPS1)
PANU100998090(CPS1)
TGE112636758(CPS1)
RRO104655495(CPS1)
RBB108540334(CPS1)
TFN117094576(CPS1)
PTEH111548015(CPS1)
CJC100402602(CPS1)
SBQ101050943(CPS1)
CSYR103265850(CPS1)
MMUR105863911(CPS1)
OGA100951321(CPS1)
MMU227231(Cps1)
MCAL110289777(Cps1)
MPAH110322614(Cps1)
RNO497840(Cps1)
MCOC116089210(Cps1)
MUN110539643
CGE100760467(Cps1)
MAUA101832270(Cps1)
PLEU114693613(Cps1)
MORG121433434(Cps1)
NGI103744467(Cps1)
HGL101696508(Cps1)
CPOC100723563(Cps1)
CCAN109699651(Cps1)
DORD105995361(Cps1)
DSP122107420(Cps1)
NCAR124979792
OCU100303765(CPS1)
OPI101527604(CPS1)
TUP102491899(CPS1)
CFA488506(CPS1)
VVP112922588(CPS1)
VLG121480750(CPS1)
AML100466842(CPS1)
UMR103659822(CPS1)
UAH113250504(CPS1)
UAR123798268(CPS1)
ELK111156350
LLV125095184
MPUF101674281(CPS1)
ORO101371212(CPS1)
EJU114215934(CPS1)
ZCA113920213(CPS1)
MLX118012083(CPS1)
FCA101099824(CPS1)
PYU121031013(CPS1)
PBG122481726(CPS1)
PTG102968283(CPS1)
PPAD109265124(CPS1)
AJU106984775(CPS1)
HHV120243755(CPS1)
BTA510506(CPS1)
BOM102267831(CPS1)
BIU109570387(CPS1)
BBUB102415970(CPS1)
CHX102179612(CPS1)
OAS101107584(CPS1)
ODA120854532(CPS1)
CCAD122426621(CPS1)
SSC100157716(CPS1)
CFR102516394(CPS1)
CBAI105072943(CPS1)
CDK105101586(CPS1)
VPC102537219(CPS1)
BACU103003987(CPS1)
LVE103079188(CPS1)
OOR101279675(CPS1)
DLE111171789(CPS1)
PCAD102973240(CPS1)
PSIU116756616(CPS1)
ECB100050116(CPS1)
EPZ103554808(CPS1)
EAI106843053(CPS1)
MYB102262103(CPS1)
MYD102756563(CPS1)
MMYO118661701(CPS1)
MLF102436612(CPS1)
MNA107524839(CPS1)
PKL118709020(CPS1)
HAI109390430(CPS1)
DRO112307671(CPS1)
SHON118984231(CPS1)
AJM119044341(CPS1)
PDIC114494376(CPS1)
PHAS123828195(CPS1)
MMF118625808(CPS1)
RFQ117025658(CPS1)
PALE102895317(CPS1)
PGIG120587605(CPS1)
PVP105291095(CPS1)
RAY107517643(CPS1)
MJV108396990(CPS1)
TOD119256167(CPS1)
SARA101556847(CPS1)
LAV100669826(CPS1)
TMU101359193
DNM101436387(CPS1)
MDO100015090(CPS1)
GAS123242723(CPS1)
SHR100929825(CPS1)
PCW110223299(CPS1)
OAA100077228(CPS1)
GGA428994(CPS1)
PCOC116235914(CPS1)
MGP100539661(CPS1)
CJO107316483(CPS1)
NMEL110399288(CPS1)
APLA101797791(CPS1)
ACYG106034508(CPS1)
AFUL116490822(CPS1)
TGU100221389(CPS1)
LSR110471694(CPS1)
SCAN103814751(CPS1)
PMOA120511507(CPS1)
OTC121339687(CPS1)
PRUF121364445(CPS1)
GFR102044867(CPS1)
FAB101816308(CPS1)
PHI102105821(CPS1)
PMAJ107207199(CPS1)
CCAE111931881(CPS1)
CCW104694540(CPS1)
ETL114059416(CPS1)
ZAB102066900(CPS1)
FPG101910440(CPS1)
FCH102046837(CPS1)
CLV102091543(CPS1)
EGZ104124302(CPS1)
NNI104009921(CPS1)
ACUN113482015(CPS1)
TALA104368683(CPS1)
PADL103921645(CPS1)
ACHC115343371(CPS1)
AAM106492382(CPS1)
AROW112977009(CPS1)
NPD112955714(CPS1)
DNE112996164(CPS1)
ASN102386005(CPS1)
AMJ106736782(CPS1)
CPOO109323109(CPS1)
GGN109301987(CPS1)
PSS102462440(CPS1)
CMY102943091(CPS1)
CPIC101942404(CPS1)
TST117884622(CPS1)
CABI116816082(CPS1)
MRV120374564(CPS1)
ACS100562333(cps1)
PVT110072386(CPS1)
SUND121919956(CPS1)
PBI103061484(CPS1)
PMUR107301875(CPS1)
TSR106557156(CPS1)
PGUT117674372(CPS1)
VKO123024301(CPS1)
PMUA114583459(CPS1)
ZVI118096710(CPS1)
XLA733340(cps1.L)
XTR100158506(cps1)
NPR108794421(CPS1)
RTEM120943863(CPS1)
BBUF121007628(CPS1)
BGAR122945871(CPS1)
DRE555623(cps1)
SRX107741925
SANH107660611
SGH107555908(cps1)
CAUA113108375(cps1)
PPRM120461485 120461522(cps1)
MAMB125270576(cps1)
IPU108266807(cps1)
PHYP113526109(cps1)
SMEO124383048(cps1)
TFD113648186(cps1)
AMEX103026142(cps1)
EEE113589154(cps1)
TRU101074466(cps1)
TNGGSTEN00004415G001
LCO104929821(cps1)
NCC104953308(cps1)
CGOB115026190(cps1)
ELY117251123(cps1)
PLEP121949412(cps1)
SLUC116037726(cps1) 116050235
ECRA117952874(cps1)
PFLV114563820(cps1)
GAT120834388(cps1)
PPUG119229014(cps1)
MSAM119903960(cps1)
CUD121523069(cps1)
ALAT119025840(cps1)
MZE101479159(cps1)
ONL100696002(cps1)
OAU116312077(cps1)
OLA101164854(cps1)
OML112155538(cps1)
XMA102232024(cps1)
XCO114147479(cps1)
XHE116723745(cps1)
PRET103461545(cps1)
PFOR103141450(cps1)
PLAI106948981(cps1)
PMEI106918783(cps1)
GAF122839359(cps1)
CVG107088030(cps1)
CTUL119798621(cps1)
GMU124871823(cps1)
NFU107396242(cps1)
KMR108240446(cps1)
ALIM106519746(cps1)
NWH119418844(cps1)
AOCE111584991(cps1)
CSEM103392237(cps1)
POV109631134(cps1)
SSEN122765088(cps1)
HHIP117755012(cps1)
HSP118119733(cps1)
LCF108892757(cps1)
SDU111218322(cps1)
SLAL111662716(cps1)
XGL120801664(cps1)
HCQ109531202(cps1)
BPEC110156044(cps1)
BSPL114847503(cps1)
SASA106586447(cps1)
OTW112238893(cps1)
OMY100136758(cps1)
OGO124044011(cps1)
ONE115111283(cps1)
SALP112068953(cps1)
SNH120058044(cps1)
ELS105008497(cps1)
SFM108922278(cps1)
AANG118224050(cps1)
LOC102687173(cps1)
PSPA121323136(cps1)
ARUT117412900(cps1)
LCM102363877(CPS1)
CMK103182939(cps1)
RTP109937803(cps1)
BFO118405554
BBEL109465412
SCLV120327643 120328367
SPU584369
APLC110989285
SKO100367850
LGILOTGIDRAFT_137874
PCAN112558598
BGT106078424
GAE121387415
HRF124119867
HRJ124262534
OBI106879763
OSN115215632
LAK106172324 106173741
NVE5504557 5515702
EPA110246605
ATEN116288266
ADF107350432 107350434
AMIL114968085
PDAM113673921
SPIS111323363
DGT114517513
XEN124452820
PTIPHATRDRAFT_24195
FCYFRACYDRAFT_169332(Fc_169332)
TPSTHAPSDRAFT_40323
AAFAURANDRAFT_20552(CPS1)
PIFPITG_03098
PSOJPHYSODRAFT_348996
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

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