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2 ATP + L-Gln + HCO3-
2 ADP + phosphate + L-Glu + carbamoyl phosphate
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
2 ATP + NH3 + CO2 + H2O
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
ATP + carbamate
ADP + carbamoyl phosphate
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoylphosphate
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
additional information
?
-
2 ATP + L-Gln + HCO3-
2 ADP + phosphate + L-Glu + carbamoyl phosphate
-
-
-
?
2 ATP + L-Gln + HCO3-
2 ADP + phosphate + L-Glu + carbamoyl phosphate
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
plays a key role in the regulation of the urea cycle
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
primary ammonia-detoxifying enzyme
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
enzyme is involved in arginine and urea synthesis
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
primary ammonia-detoxifying enzyme
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
-
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
first enzyme involved in the biosynthesis of urea in the liver ureotelic animals
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
enzyme of urea cycle
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of MgATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of CoATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of MgATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of MnATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of MgATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
ATP in form of CoATP2-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
2 ATP + NH4+ + HCO3-
2 ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + carbamate
ADP + carbamoyl phosphate
-
-
r
ATP + carbamate
ADP + carbamoyl phosphate
-
-
-
r
ATP + carbamate
ADP + carbamoyl phosphate
-
-
-
r
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
first step of the both the urea cycle and pyrimidine nucleotide biosynthesis patways
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
the enzyme T1405N genotype influences nitric oxide production as well as vascular smooth muscle reactivity
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
CPS1 is a rate-limiting enzyme in the urea cycle
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
three step reaction
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
three step reaction
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
the first and rate-limiting step of the urea synthesis
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
enzyme of the urea cycle
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
CPS is the rate-determining enzyme of the urea cycle and catalyzes the conversion of ammonia and bicarbonate to carbamoylphosphate in the periportal and intermediate zones of a liver lobule, overview. The enzyme is responsible for ammonia elimination
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
-
?
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
the product carbamoyl phosphate is used in the biosynthesis of pyrimidines, arginine, and urea
-
ir
ATP + NH3 + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
the product carbamoylphosphate is used in the biosynethsis of pyrimidines, arginine, and urea
-
ir
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
Frog
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
no activity with Gln
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
no activity with Gln
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
no activity with Gln
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
no activity with Gln
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
?
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
-
?
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
the human enzyme removes excess, potentially neurotoxic ammonia via the urea cycle in a tightly regulated manner that avoids depletion of the ammonia pool, and shows inability to use glutamine as an alternative nitrogen donor
-
-
?
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
synthesis of carbamoyl phosphate requires coordination of two hCPS active sites with ATP cleavage occurring at duplicated ATP grasp domains B and C
-
-
?
ATP + NH4+ + HCO3- + H2O
ADP + phosphate + carbamoyl phosphate
-
-
-
?
additional information
?
-
-
Rana cancrivora up-regulates hepatic carbamoyl phosphate synthetase I activity and tissue osmolyte levels in response to increased salinity
-
-
?
additional information
?
-
-
polymorphism of carbamyl phosphate synthetase I, certain carbamyl phosphate synthetase I alleles are associated with clinical outcome
-
-
?
additional information
?
-
the enzyme might play an important role in spermiogenesis
-
-
?
additional information
?
-
-
the enzyme might play an important role in spermiogenesis
-
-
?
additional information
?
-
severe hyperammonaemia and high mortality are observed in hCPS deficiency, a rare autosomal disease
-
-
?
additional information
?
-
-
severe hyperammonaemia and high mortality are observed in hCPS deficiency, a rare autosomal disease
-
-
?
additional information
?
-
-
this enzyme is unable to bind and utilize L-glutamine
-
?
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0.39
ammonia
-
wild-type enzyme, comparison to native and mutant enzyme
0.29 - 0.44
N-acetylglutamate
additional information
additional information
-
0.0372
ATP
pH 8.0, deletion mutant lacking the last 13 residues of the carboxyl end
0.06
ATP
pH 8.0, recombinant enzyme
0.27
ATP
-
wild-type enzyme, comparison to native and mutant enzyme
0.43
ATP
mutant enzyme Y389C, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.45
ATP
mutant enzyme G1376S, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.45
ATP
mutant enzyme T344A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.47
ATP
recombinant wild type enzyme, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.55
ATP
pH 7.6, 37°C, wild-type enzyme
0.57
ATP
mutant enzyme N355D, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.6 - 1
ATP
at pH 8.0 and 37°C
0.62
ATP
mutant enzyme T1443A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.68
ATP
pH 7.6, 37°C, mutant T1406D
0.9
ATP
mutant enzyme A1378T, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1.92
ATP
mutant enzyme T544M, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
2.3
CO2
-
NaHCO3, 37°C, pH 8
7.2
CO2
-
NaHCO3, 70°C, pH 7.5
1 - 4.5
HCO3-
at pH 8.0 and 37°C
2.44
HCO3-
pH 7.6, 37°C, wild-type enzyme
2.49
HCO3-
pH 7.6, 37°C, mutant T1406D
3.6
HCO3-
mutant enzyme T1443A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4
HCO3-
recombinant wild type enzyme, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4.2
HCO3-
mutant enzyme Y389C, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4.3
HCO3-
mutant enzyme A1378T, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4.3
HCO3-
mutant enzyme T344A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4.5
HCO3-
mutant enzyme G1376S, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
4.6
HCO3-
mutant enzyme N355D, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
242
HCO3-
mutant enzyme T544M, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
0.29
N-acetylglutamate
pH 7.6, 37°C, wild-type enzyme
0.44
N-acetylglutamate
pH 7.6, 37°C, mutant T1406D
0.038
NH3
-
-
0.35
NH4+
pH 7.6, 37°C, wild-type enzyme
0.58
NH4+
pH 7.6, 37°C, mutant T1406D
0.9
NH4+
mutant enzyme A1378T, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1
NH4+
mutant enzyme N355D, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1
NH4+
mutant enzyme T1443A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1
NH4+
recombinant wild type enzyme, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1.1
NH4+
mutant enzyme G1376S, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1.1
NH4+
mutant enzyme Y389C, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1.2
NH4+
mutant enzyme T344A, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
1.3
NH4+
mutant enzyme T544M, in 50 mM glycyl-glycine pH 7.4, 70 mM KCl, 1 mM dithiothreitol, 20 mM MgSO4, at 37°C
15.6
NH4+
at pH 8.0 and 37°C
additional information
additional information
-
kinetic parameters for utilization of ATP and ADP by mutant enzymes
-
additional information
additional information
kinetics of wild-type and mutant enzymes, overview. The Km of the enzyme for ammonia is 100fold lower than that for CPSs that also use glutamine, EC 6.3.5.5
-
additional information
additional information
-
kinetics of wild-type and mutant enzymes, overview. The Km of the enzyme for ammonia is 100fold lower than that for CPSs that also use glutamine, EC 6.3.5.5
-
additional information
additional information
kinetics, the Km of the enzyme for ammonia is 100fold lower than that for CPSs that also use glutamine, EC 6.3.5.5, kinetics for bicarbonate-dependent ATPase and carbamoyl phosphate-dependent ATP synthesis reactions, overview
-
additional information
additional information
-
kinetics, the Km of the enzyme for ammonia is 100fold lower than that for CPSs that also use glutamine, EC 6.3.5.5, kinetics for bicarbonate-dependent ATPase and carbamoyl phosphate-dependent ATP synthesis reactions, overview
-
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Britton, H.G.; Rubio, V.
Carbamoyl-phosphate synthetase I. Kinetic of binding and dissociation of acetylglutamate and of activation and deactivation
Eur. J. Biochem.
171
615-622
1988
Rattus norvegicus
brenda
Lund, P.; Wiggins, D.
Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate
Biochem. J.
243
273-276
1987
Rattus norvegicus
brenda
Cohen, N.S.; Kyan, F.S.; Kyan, S.S.; Cheung, C.W.; Raijman, L.
The apparent Km of ammonia for carbamoyl phosphate synthetase (ammonia) in situ
Biochem. J.
229
205-211
1988
Rattus norvegicus
brenda
Guadalajara, A.M.; Rubio, V.; Grisolia, S.
Inactivation of carbamoyl phosphate synthetase (ammonia) by elastase as a probe to investigate binding of the substrates
Biochem. Biophys. Res. Commun.
117
238-244
1983
Frog, Mesocricetus auratus, Rattus norvegicus
brenda
Lof, C.; Cohen, M.; Vermeulen, L.P.; van Roermund, C.W.T.; Wanders, R.J.A.; Meijer, A.J.
Properties of carbamoyl-phosphate synthetase (ammonia) in rat-liver mitochondria made permeable with toluene
Eur. J. Biochem.
135
251-258
1983
Rattus norvegicus
brenda
McIntyre, P.; Hoogenraad, N.
Isolation of an immunologically pure preparation of carabamylphosphate synthetase (ammonia) using chromatofocusing
FEBS Lett.
135
65-69
1981
Rattus norvegicus
brenda
Meijer, A.J.; Woerkom, G.M.; Steinman, R.; Williamson, J.R.
Inhibition by Ca2+ of carbamoylphosphate synthetase (ammonia)
J. Biol. Chem.
256
3443-3446
1981
Rattus norvegicus
brenda
Cohen, N.S.; Raijman, L.
The apparent Km of ATPMg for carbamoyl phosphate synthetase (ammonia) in situ
J. Biol. Chem.
255
3352-3357
1980
Rattus norvegicus
brenda
Raijman, L.; Jones, M.E.
Purification, composition, and some properties of rat liver carbamyl phosphate synthetase (ammonia)
Arch. Biochem. Biophys.
175
270-278
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