6.3.4.16: carbamoyl-phosphate synthase (ammonia)
This is an abbreviated version!
For detailed information about carbamoyl-phosphate synthase (ammonia), go to the full flat file.
Word Map on EC 6.3.4.16
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6.3.4.16
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ornithine
-
pyrimidine
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transcarbamylase
-
hepatocytes
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hyperammonemia
-
n-acetylglutamate
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bicarbonate
-
carbamoyltransferase
-
citrulline
-
arginase
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dihydroorotase
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glutaminase
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atcase
-
ump
-
amidotransferase
-
glutamine-dependent
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uracil
-
periportal
-
ureotelic
-
mgatp
-
carboxykinase
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ureagenesis
-
orotate
-
hepatocyte-specific
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acidurias
-
dhoase
-
squalus
-
l-glutamine
-
acanthias
-
bicarbonate-dependent
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2.1.3.2
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acivicin
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intramitochondrial
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zonation
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nh3
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ammonia-dependent
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pericentral
-
coma
-
urea-cycle
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biotechnology
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neonatal-onset
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citrullinemia
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medicine
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argininosuccinase
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elasmobranch
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analysis
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perivenous
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dogfish
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glucocorticosteroids
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5-phosphoribosyl
- 6.3.4.16
- ornithine
- pyrimidine
-
transcarbamylase
- hepatocytes
-
hyperammonemia
- n-acetylglutamate
- bicarbonate
-
carbamoyltransferase
- citrulline
- arginase
- dihydroorotase
- glutaminase
- atcase
- ump
-
amidotransferase
-
glutamine-dependent
- uracil
-
periportal
-
ureotelic
- mgatp
-
carboxykinase
-
ureagenesis
- orotate
-
hepatocyte-specific
- acidurias
- dhoase
-
squalus
- l-glutamine
- acanthias
-
bicarbonate-dependent
-
2.1.3.2
- acivicin
-
intramitochondrial
-
zonation
- nh3
-
ammonia-dependent
-
pericentral
- coma
-
urea-cycle
- biotechnology
-
neonatal-onset
- citrullinemia
- medicine
- argininosuccinase
-
elasmobranch
- analysis
-
perivenous
- dogfish
-
glucocorticosteroids
-
5-phosphoribosyl
Reaction
2 ATP
+
Synonyms
arginine-specific CPS, CarB, carbamate kinase-like carbamoyl phosphate synthetase, carbamate kinase-like carbamoyl-phosphate synthetase, carbamoyl phosphate synthase-1, carbamoyl phosphate synthetase, carbamoyl phosphate synthetase 1, carbamoyl phosphate synthetase I, carbamoyl phosphate synthetase-1, carbamoyl-phosphate synthetase, carbamoyl-phosphate synthetase 1, Carbamoyl-phosphate synthetase I, Carbamoylphosphate synthase, Carbamoylphosphate synthase (ammonia), Carbamoylphosphate synthetase, Carbamoylphosphate synthetase (ammonia), Carbamoylphosphate synthetase I, carbamoylphosphate synthetase-I, Carbamyl phosphate synthase I, carbamyl phosphate synthetase I, carbamyl-phosphate synthetase I, Carbamylphosphate synthetase, carbamylphosphate synthetase 1, Carbamylphosphate synthetase I, carbmoylphosphate synthetase, Carbon-dioxide-ammonia ligase, Carbonate kinase (phosphorylating), CK-like CPS, CPS, CPS 1, CPS I, CPS I-like (ammonia- and N-acetyl-L-glutamate-dependent), CPS-1, CPS-I, CPS1, CPSase, CPSase I, CPSI, EC 2.7.2.5, mitochondrial carbamoylphosphate synthetase I, MS-s, Msm_0361, p165, urea-specific CPS
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6.3.4.16 carbamoyl-phosphate synthase (ammonia)Advanced search results
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results (Substrates Products
Substrates Products on EC 6.3.4.16 - carbamoyl-phosphate synthase (ammonia)
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
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
?
-
plays a key role in the regulation of the urea cycle
-
-
?
2 ATP + NH3 + CO2 + H2O
?
-
first enzyme involved in the biosynthesis of urea in the liver ureotelic animals
-
-
?
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
-
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 + 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
-
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
-
the first and rate-limiting step of the urea synthesis
-
?
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
-
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
-
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
-
no activity with Gln
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
-
-
-
?
ATP + NH4+ + CO2 + H2O
ADP + phosphate + carbamoylphosphate
-
no activity with Gln
-
?
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
-
-
?
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
-
?
