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)
NLE: 100596216(CPS1)
HMH: 116810358(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)
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)
ODA: 120854532(CPS1)
CCAD: 122426621(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)
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)
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)
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)
ETL: 114059416(CPS1)
ZAB: 102066900(CPS1)
ACHL: 103807118(CPS1)
SVG: 106856551(CPS1)
MMEA: 130588855(CPS1)
HRT: 120754985(CPS1)
FPG: 101910440(CPS1)
FCH: 102046837(CPS1)
CLV: 102091543(CPS1)
EGZ: 104124302(CPS1)
NNI: 104009921(CPS1)
PCRI: 104030248(CPS1)
PLET: 104627545(CPS1)
EHS: 104510972
PCAO: 104045913(CPS1)
ACUN: 113482015(CPS1)
TALA: 104368683(CPS1)
PADL: 103921645(CPS1)
AFOR: 103900404(CPS1)
ACHC: 115343371(CPS1)
HALD: 104321187
HLE: 104841537(CPS1)
AGEN: 126039478
GCL: 127018302
CCRI: 104161143(CPS1)
CMAC: 104477513(CPS1)
MUI: 104538785(CPS1)
BREG: 104643520(CPS1)
FGA: 104075389(CPS1)
GSTE: 104254124(CPS1)
LDI: 104350520(CPS1)
MNB: 103771914(CPS1)
OHA: 104338581(CPS1)
NNT: 104405431(CPS1)
SHAB: 115608976(CPS1)
DPUB: 104299635
PGUU: 104466714(CPS1)
ACAR: 104531757(CPS1)
CPEA: 104388101(CPS1)
AVIT: 104278583(CPS1)
CVF: 104288744(CPS1)
RTD: 128912324(CPS1)
CUCA: 104064581(CPS1)
TEO: 104379491(CPS1)
BRHI: 104497824(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
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)
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)
MSAM: 119903960(cps1)
SCHU: 122885023(cps1)
CUD: 121523069(cps1)
ALAT: 119025840(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)
MCEP: 125018029(cps1)
CSEM: 103392237(cps1)
POV: 109631134(cps1)
SSEN: 122765088(cps1)
HHIP: 117755012(cps1)
HSP: 118119733(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)
CPLA: 122551475(cps1)
HOC: 132817290(cps1)
LERI: 129698851(cps1)
BFO: 118405554
BBEL: 109465412
SPU: 584369
LPIC: 129257812
APLC: 110989285
AJC: 117122950
SKO: 100367850
PCAN: 112558598
BGT: 106078424
GAE: 121387415
HRF: 124119867
HRJ: 124262534
OBI: 106879763
OSN: 115215632
EPA: 110246605
ATEN: 116288266
AMIL: 114968085
PDAM: 113673921
SPIS: 111323363
DGT: 114517513
XEN: 124452820
FCY: FRACYDRAFT_169332(Fc_169332)
AAF: AURANDRAFT_20552(CPS1)
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]

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