KEGG ORTHOLOGY: K01948

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

LGF:	123599410(CPS1)

AJU:

106984775

PTG:	102968283(CPS1)

PPAD:

109265124(CPS1)

PUC:

125911234

PLEZ:

122226581(CPS1)

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)

LALB:

132521659(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)

MYUM:

139014979(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)

ATRC:

139593478(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)

CCIC:

134052319(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)

CRID:

134518448(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)

PWL:	138285561(CPS1)

MUO:	115473875(CPS1)

GSH:	117361602(CPS1)

DRE:	555623(cps1)

SRX:

107741925

SANH:

107660611

SGH:	107555908(cps1)

CCAR:

109083580(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)

CGAR:

128524530(cps1)

NGRA:

132891446(cps1)

AMEX:

103026142(cps1)

CMAO:

118812551(cps1)

EEE:	113589154(cps1)

CHAR:

105908013(cps1)

SPIL:

134078118(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)

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)

SSOE:

131455283(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)

ENY:	140359872(cps1)

BSPL:

114847503(cps1)

SJO:	128368364(cps1)

SSCO:

133992618(cps1)

GMH:	115533599(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)

OCLA:

139401342

SALP:

112068953(cps1)

SNH:	120058044(cps1)

CCLU:

121536995(cps1)

ELS:	105008497(cps1)

OEP:	134016986(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:

BBEL:

SCLV:

SPU:

LPIC:

AJJ:

APLC:

ARUN:

AAMU:

AFIL:

AJC:

SKO:

PFLL:

LGI:	LOTGIDRAFT_137874

PCAN:

LSAX:

BGT:

GAE:

HRF:

HRJ:

HASI:

HCRA:

OBI:

OSN:

LJP:

LAK:	106172324 106173741

NVE:	5504557 5515702

EPA:

110246605

ATEN:

116288266

ADF:	107350432 107350434

AMIL:

MCAZ:

MFOO:

PDAM:

SPIS:

DGT:

XEN:

RES:

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

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

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