KEGG   ORTHOLOGY: K01948
Entry
K01948                      KO                                     

Name
CPS1
Definition
carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
Pathway
ko00220  Arginine biosynthesis
ko00250  Alanine, aspartate and glutamate metabolism
ko00910  Nitrogen metabolism
ko01100  Metabolic pathways
ko01120  Microbial metabolism in diverse environments
ko01200  Carbon metabolism
ko01230  Biosynthesis of amino acids
Module
M00029  Urea cycle
Disease
H00164  Carbamoyl phosphate synthetase I deficiency
H01398  Primary hyperammonemic disorders (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
RN: R00149
GO: 0004087
Genes
HSA: 1373(CPS1)
PTR: 738670(CPS1)
PPS: 100984596(CPS1)
GGO: 101130709(CPS1)
PON: 100442727(CPS1)
NLE: 100596216(CPS1)
MCC: 711806(CPS1)
MCF: 102139369(CPS1)
CSAB: 103217763(CPS1)
RRO: 104655495(CPS1)
RBB: 108540334(CPS1)
CJC: 100402602(CPS1)
SBQ: 101050943(CPS1)
MMU: 227231(Cps1)
MCAL: 110289777(Cps1)
MPAH: 110322614(Cps1)
RNO: 497840(Cps1)
MUN: 110539643
CGE: 100760467(Cps1)
NGI: 103744467(Cps1)
HGL: 101696508(Cps1)
CCAN: 109699651(Cps1)
OCU: 100303765(CPS1)
TUP: 102491899(CPS1)
CFA: 488506(CPS1)
VVP: 112922588(CPS1)
AML: 100466842(CPS1)
UMR: 103659822(CPS1)
UAH: 113250504(CPS1)
ORO: 101371212(CPS1)
ELK: 111156350
FCA: 101099824(CPS1)
PTG: 102968283(CPS1)
PPAD: 109265124(CPS1)
AJU: 106984775(CPS1)
BTA: 510506(CPS1)
BOM: 102267831(CPS1)
BIU: 109570387(CPS1)
BBUB: 102415970(CPS1)
CHX: 102179612(CPS1)
OAS: 101107584(CPS1)
SSC: 100157716(CPS1)
CFR: 102516394(CPS1)
CDK: 105101586(CPS1)
BACU: 103003987(CPS1)
LVE: 103079188(CPS1)
OOR: 101279675(CPS1)
DLE: 111171789(CPS1)
PCAD: 102973240(CPS1)
ECB: 100050116(CPS1)
EPZ: 103554808(CPS1)
EAI: 106843053(CPS1)
MYB: 102262103(CPS1)
MYD: 102756563(CPS1)
MNA: 107524839(CPS1)
HAI: 109390430(CPS1)
DRO: 112307671(CPS1)
PALE: 102895317(CPS1)
RAY: 107517643(CPS1)
MJV: 108396990(CPS1)
LAV: 100669826(CPS1)
TMU: 101359193
MDO: 100015090(CPS1)
SHR: 100929825(CPS1)
PCW: 110223299(CPS1)
OAA: 100077228(CPS1)
GGA: 428994(CPS1)
MGP: 100539661(CPS1)
CJO: 107316483(CPS1)
NMEL: 110399288(CPS1)
APLA: 101797791(CPS1)
ACYG: 106034508(CPS1)
TGU: 100221389(CPS1)
LSR: 110471694(CPS1)
SCAN: 103814751(CPS1)
GFR: 102044867(CPS1)
FAB: 101816308(CPS1)
PHI: 102105821(CPS1)
PMAJ: 107207199(CPS1)
CCAE: 111931881(CPS1)
CCW: 104694540(CPS1)
ETL: 114059416(CPS1)
FPG: 101910440(CPS1)
FCH: 102046837(CPS1)
CLV: 102091543(CPS1)
EGZ: 104124302(CPS1)
NNI: 104009921(CPS1)
ACUN: 113482015(CPS1)
PADL: 103921645(CPS1)
AAM: 106492382(CPS1)
ASN: 102386005(CPS1)
AMJ: 106736782(CPS1)
PSS: 102462440(CPS1)
CMY: 102943091(CPS1)
CPIC: 101942404(CPS1)
ACS: 100562333(cps1)
PVT: 110072386(CPS1)
PBI: 103061484(CPS1)
PMUR: 107301875(CPS1)
TSR: 106557156(CPS1)
PMUA: 114583459(CPS1)
XLA: 733340(cps1.L)
XTR: 100158506(cps1)
NPR: 108794421(CPS1)
DRE: 555623(cps1)
SRX: 107741925
SANH: 107660611
SGH: 107555908(cps1)
IPU: 108266807(cps1)
PHYP: 113526109(cps1)
AMEX: 103026142(cps1)
EEE: 113589154(cps1)
TRU: 101074466(cps1)
LCO: 104929821(cps1)
NCC: 104953308(cps1)
MZE: 101479159(cps1)
ONL: 100696002(cps1)
OLA: 101164854(cps1)
XMA: 102232024(cps1)
XCO: 114147479(cps1)
PRET: 103461545(cps1)
CVG: 107088030(cps1)
NFU: 107396242(cps1)
KMR: 108240446(cps1)
ALIM: 106519746(cps1)
AOCE: 111584991(cps1)
CSEM: 103392237(cps1)
POV: 109631134(cps1)
LCF: 108892757(cps1)
SDU: 111218322(cps1)
SLAL: 111662716(cps1)
HCQ: 109531202(cps1)
BPEC: 110156044(cps1)
SASA: 106586447(cps1)
OTW: 112238893(cps1)
SALP: 112068953(cps1)
ELS: 105008497(cps1)
SFM: 108922278(cps1)
LCM: 102363877(CPS1)
CMK: 103182939(cps1)
RTP: 109937803(cps1)
SPU: 584369
APLC: 110989285
SKO: 100367850
PCAN: 112558598
OBI: 106879763
EPA: 110246605
AMIL: 114968085
PDAM: 113673921
SPIS: 111323363
DGT: 114517513
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]

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
HSA: 1373(CPS1)
PTR: 738670(CPS1)
PPS: 100984596(CPS1)
GGO: 101130709(CPS1)
PON: 100442727(CPS1)
NLE: 100596216(CPS1)
MCC: 711806(CPS1)
MCF: 102139369(CPS1)
CSAB: 103217763(CPS1)
RRO: 104655495(CPS1)
RBB: 108540334(CPS1)
CJC: 100402602(CPS1)
SBQ: 101050943(CPS1)
MMU: 227231(Cps1)
MCAL: 110289777(Cps1)
MPAH: 110322614(Cps1)
RNO: 497840(Cps1)
MUN: 110539643
CGE: 100760467(Cps1)
NGI: 103744467(Cps1)
HGL: 101696508(Cps1)
CCAN: 109699651(Cps1)
OCU: 100303765(CPS1)
TUP: 102491899(CPS1)
CFA: 488506(CPS1)
VVP: 112922588(CPS1)
AML: 100466842(CPS1)
UMR: 103659822(CPS1)
UAH: 113250504(CPS1)
ORO: 101371212(CPS1)
ELK: 111156350
FCA: 101099824(CPS1)
PTG: 102968283(CPS1)
PPAD: 109265124(CPS1)
AJU: 106984775(CPS1)
BTA: 510506(CPS1)
BOM: 102267831(CPS1)
BIU: 109570387(CPS1)
BBUB: 102415970(CPS1)
CHX: 102179612(CPS1)
OAS: 101107584(CPS1)
SSC: 100157716(CPS1)
CFR: 102516394(CPS1)
CDK: 105101586(CPS1)
BACU: 103003987(CPS1)
LVE: 103079188(CPS1)
OOR: 101279675(CPS1)
DLE: 111171789(CPS1)
PCAD: 102973240(CPS1)
ECB: 100050116(CPS1)
EPZ: 103554808(CPS1)
EAI: 106843053(CPS1)
MYB: 102262103(CPS1)
MYD: 102756563(CPS1)
MNA: 107524839(CPS1)
HAI: 109390430(CPS1)
DRO: 112307671(CPS1)
PALE: 102895317(CPS1)
RAY: 107517643(CPS1)
MJV: 108396990(CPS1)
LAV: 100669826(CPS1)
TMU: 101359193
MDO: 100015090(CPS1)
SHR: 100929825(CPS1)
PCW: 110223299(CPS1)
OAA: 100077228(CPS1)
GGA: 428994(CPS1)
MGP: 100539661(CPS1)
CJO: 107316483(CPS1)
NMEL: 110399288(CPS1)
APLA: 101797791(CPS1)
ACYG: 106034508(CPS1)
TGU: 100221389(CPS1)
LSR: 110471694(CPS1)
SCAN: 103814751(CPS1)
GFR: 102044867(CPS1)
FAB: 101816308(CPS1)
PHI: 102105821(CPS1)
PMAJ: 107207199(CPS1)
CCAE: 111931881(CPS1)
CCW: 104694540(CPS1)
ETL: 114059416(CPS1)
FPG: 101910440(CPS1)
FCH: 102046837(CPS1)
CLV: 102091543(CPS1)
EGZ: 104124302(CPS1)
NNI: 104009921(CPS1)
ACUN: 113482015(CPS1)
PADL: 103921645(CPS1)
AAM: 106492382(CPS1)
ASN: 102386005(CPS1)
AMJ: 106736782(CPS1)
PSS: 102462440(CPS1)
CMY: 102943091(CPS1)
CPIC: 101942404(CPS1)
ACS: 100562333(cps1)
PVT: 110072386(CPS1)
PBI: 103061484(CPS1)
PMUR: 107301875(CPS1)
TSR: 106557156(CPS1)
PMUA: 114583459(CPS1)
XLA: 733340(cps1.L)
XTR: 100158506(cps1)
NPR: 108794421(CPS1)
DRE: 555623(cps1)
SRX: 107741925
SANH: 107660611
SGH: 107555908(cps1)
IPU: 108266807(cps1)
PHYP: 113526109(cps1)
AMEX: 103026142(cps1)
EEE: 113589154(cps1)
TRU: 101074466(cps1)
LCO: 104929821(cps1)
NCC: 104953308(cps1)
MZE: 101479159(cps1)
ONL: 100696002(cps1)
OLA: 101164854(cps1)
XMA: 102232024(cps1)
XCO: 114147479(cps1)
PRET: 103461545(cps1)
CVG: 107088030(cps1)
NFU: 107396242(cps1)
KMR: 108240446(cps1)
ALIM: 106519746(cps1)
AOCE: 111584991(cps1)
CSEM: 103392237(cps1)
POV: 109631134(cps1)
LCF: 108892757(cps1)
SDU: 111218322(cps1)
SLAL: 111662716(cps1)
HCQ: 109531202(cps1)
BPEC: 110156044(cps1)
SASA: 106586447(cps1)
OTW: 112238893(cps1)
SALP: 112068953(cps1)
ELS: 105008497(cps1)
SFM: 108922278(cps1)
LCM: 102363877(CPS1)
CMK: 103182939(cps1)
RTP: 109937803(cps1)
SPU: 584369
APLC: 110989285
SKO: 100367850
PCAN: 112558598
OBI: 106879763
EPA: 110246605
AMIL: 114968085
PDAM: 113673921
SPIS: 111323363
DGT: 114517513
FCY: FRACYDRAFT_169332(Fc_169332)
AAF: AURANDRAFT_20552(CPS1)
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

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
Orthology
K01948  carbamoyl-phosphate synthase (ammonia) [EC:6.3.4.16]
Other DBs
RHEA: 10627

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