KEGG ENZYME: 6.3.4.16

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

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)

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)

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)

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)

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)

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)

TRU:	101074466(cps1)

TFS:	130529796(cps1)

TNG:	GSTEN00004415G001

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)

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)

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)

SASA:

106586447(cps1)

STRU:

115160979(cps1)

OTW:	112238893(cps1)

OMY:	100136758(cps1)

OGO:	124044011(cps1)

ONE:	115111283(cps1)

OKI:	109868274(cps1)

OKE:	118366886(cps1)

SALP:

112068953(cps1)

SNH:	120058044(cps1)

CCLU:

121536995(cps1)

ELS:	105008497(cps1)

SFM:	108922278(cps1)

AANG:

118224050(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

SCLV:

120327643 120328367

SPU:

584369

LPIC:

129257812

APLC:

110989285

ARUN:

117291237

AJC:

117122950

SKO:

100367850

LGI:	LOTGIDRAFT_137874

PCAN:

112558598

BGT:

106078424

GAE:

121387415

HRF:

124119867

HRJ:

124262534

HASI:

137298909

OBI:

106879763

OSN:

115215632

LJP:

135464549

LAK:	106172324 106173741

NVE:	5504557 5515702

EPA:

110246605

ATEN:

116288266

ADF:	107350432 107350434

AMIL:

114968085

MCAZ:

138049324

PDAM:

113673921

SPIS:

111323363

DGT:

114517513

XEN:

124452820

RES:

135681610

PTI:	PHATRDRAFT_24195

FCY:	FRACYDRAFT_169332(Fc_169332)

TPS:	THAPSDRAFT_40323

AAF:	AURANDRAFT_20552(CPS1)

PIF:	PITG_03098

PSOJ:

PHYSODRAFT_348996

BLAC:

94352586(CCR75_008868)

SPAR:

SPRG_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

DBGET integrated database retrieval system