Carbamoyl phosphate synthase ammonia. Medical search. Frequent questions

Mammalian carbamyl phosphate synthetase (CPS). (wikipedia.org)
also at this step, N-acetylglutamate exerts its regulatory control on the mediating enzyme, carbamyl phosphate synthetase (CPS). (medscape.com)

Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ammonia (EC 6.3.4.16) and bicarbonate. (wikipedia.org)
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. (genetex.com)
The main uses of ammonia are in the production of fertilizers , explosives, and synthesis of organonitrogen compounds. (wikidoc.org)
Tm can sustain high levels of reduced glutathione and timely remove intracellular reactive oxygen species (ROS), through high expression of gluconeogenesis pathway key rate-limiting enzyme phosphoenolpyruvate carboxyl kinase PCK1 promoting the synthesis of glycogen, meanwhile pentose phosphate pathway producing prototype NADPH. (csi.org.cn)
This provides the ammonium ion used in the initial synthesis of carbamoyl phosphate. (wikidoc.org)
The synthesis of carbamoyl phosphate and the urea cycle are dependent on the presence of NAcGlu , which allosterically activates CPS1. (wikidoc.org)

This enzyme catalyzes the reaction of ATP and bicarbonate to produce carboxy phosphate and ADP. (wikipedia.org)
Carbamoyl phosphate synthase (CPSase) is a heterodimeric enzyme composed of a small and a large subunit (with the exception of CPSase III, which is composed of a single polypeptide that may have arisen from gene fusion of the glutaminase and synthetase domains). (wikipedia.org)
During the reaction, the arginine is attacked by the Cys residue of the enzyme establishing a tetrahedral adduct while ammonia is released. (diff.org)
Ornithine combined with carbamoyl phosphate is then converted into citrulline via the ornithine transcarbamylase (OTC) enzyme. (healthmatters.io)
The contribution of carbamoyl phosphate results from the metabolism of ammonia by the enzyme carbamoyl phosphate synthase, and if this magnesium-dependent process is impaired, ammonia buildup, or hyperammonemia can occur. (healthmatters.io)
Ornithine can also form polyamines including putrescine via the ornithine decarboxylase (ODC) enzyme, which requires pyridoxal-5-phosphate (vitamin B6) as a cofactor. (healthmatters.io)
In the absence of this enzyme, excess nitrogen accumulates in the body in the form of ammonia, which can be toxic at high levels, especially to the brain. (europa.eu)

Citrulline is generated from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. (diff.org)
Cytosolic arginine is also converted to nitric oxide and citrulline by nitric oxide synthases. (biomed.news)
Thus, both the urea and citrulline cycles are employed by CD8+ T cells to clear ammonia and enable memory development. (biomed.news)
On December 6, 2022, the journal "Nature Immunology", published the latest research paper "Ammonia detoxification promotes CD8 + T cell memory development by urea and citrulline cycles" by Prof. Huang Bo from Institute of Basic Medicine Chinese Academy of Medical Sciences / Huazhong University of Science and Technology. (csi.org.cn)
What's more, CP reacts with ornithine to generate citrulline by ornithine carbamoyl phosphotransferase (OTC). (csi.org.cn)
After citrulline enters the cytosol, arginine succinate synthase 1 (ASS1) combines to produce arginine, then arginine lyase (ASL) metabolizes it to arginine and fumaria acid, furthermore arginase 1 (ARG1) hydrolyzes arginine to ornithine and urea, at last, ornithine enters the mitochondria to complete a cycle. (csi.org.cn)

There are three different forms that serve very different functions: Carbamoyl phosphate synthetase I (mitochondria, urea cycle) Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism). (wikipedia.org)
carbamoyl phosphate synth in cytosol or mitoch? (flashcardmachine.com)

In the urea cycle, amino groups of urea are donated by carbamoyl phosphate and aspartate, while the carbon atom of urea is supplied by bicarbonate. (diff.org)
It is widely believed that ammonia is processed in the liver from the urea cycle, where hepatocyte absorbs ammonia from the Peripheral circulation and use carbamoyl phosphate synthetase 1 (CPS1) to catalyze ammonia and bicarbonate (HCO3-) and form carbamoyl phosphate (CP) in mitochondria. (csi.org.cn)

The small subunit contains the glutamine binding site and catalyses the hydrolysis of glutamine to glutamate and ammonia, which is in turn used by the large chain to synthesize carbamoyl phosphate. (wikipedia.org)

Amino acid metabolism is essential for cell survival, while the byproduct ammonia is toxic and can injure cellular longevity. (biomed.news)
In the previous study, the research team has revealed how memory T cells clear ROS, but whether CD8 + Tm cells can remove toxic ammonia by itself metabolism remains a mystery. (csi.org.cn)
To distinguish between CPS deficiency and OTC deficiency, orotic acid measurement is helpful because accumulation of carbamoyl phosphate in OTC deficiency results in its alternative metabolism to orotic acid. (msdmanuals.com)

Studies suggest that the underlying cause of the hyperammonemia is the inhibition of N -acetylglutamate synthase (NAGS) activity by free propionic acid. (medscape.com)
Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency. (carbaglu.com)

The carboxy phosphate domain found duplicated in the large subunit of CPSase is also present as a single copy in the biotin-dependent enzymes acetyl-CoA carboxylase (ACC), propionyl-CoA carboxylase (PCCase), pyruvate carboxylase (PC) and urea carboxylase. (wikipedia.org)
The large subunit in bacterial CPSase has four structural domains: the carboxy phosphate domain 1, the oligomerisation domain, the carbamoyl phosphate domain 2 and the allosteric domain. (wikipedia.org)
Carbamoylphosphate synthase (ammonia), heavy subunit. (ntu.edu.sg)

Primary urea cycle disorders (UCDs) include carbamoyl phosphate synthase (CPS) deficiency, ornithine transcarbamylase (OTC) deficiency, argininosuccinate synthetase deficiency (citrullinemia), argininosuccinate lyase deficiency (argininosuccinic aciduria), arginase deficiency (argininemia), and N -acetylglutamate synthetase (NAGS) deficiency. (msdmanuals.com)

Carbamoyl-phosphate synthase large chain [Ensembl]. (ntu.edu.sg)

Ravicti is indicated for use as adjunctive therapy for chronic management of adult and paediatric patients ≥2 months of age with urea cycle disorders (UCDs) including deficiencies of carbamoyl phosphate-synthase-I (CPS), ornithine carbamoyltransferase (OTC), argininosuccinate synthetase (ASS), argininosuccinate lyase (ASL), arginase I (ARG) and ornithine translocase deficiency hyperornithinaemia-hyperammonaemia homocitrullinuria syndrome (HHH) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. (europa.eu)

Since N -acetylglutamate (NAG) is the allosteric activator of carbamoylphosphate synthase, the entry step into the urea cycle, decreased ureagenesis occurs with accumulation of free ammonia. (medscape.com)
The result of liver failure is accumulation of nitrogenous waste, mainly ammonia, which leads to hepatic encephalopathy . (wikidoc.org)

Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation. (genetex.com)
Ornithine carbamoyltransferase deficiency is one of the inherited disorders known as 'urea cycle disorders', which cause ammonia to accumulate in the blood. (europa.eu)

Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. (wikipedia.org)

CPSase heterodimers from Escherichia coli contain two molecular tunnels: an ammonia tunnel and a carbamate tunnel. (wikipedia.org)

3-hydroxyisovaleric acid correlated positively with plasma ammonia. (biomedcentral.com)
Plasma ammonia for example, is considered the best available biochemical read-out of AMD, but does not provide insight in other pathophysiological processes that may occur during AMD. (biomedcentral.com)

Organisms that cannot easily and quickly remove ammonia usually have to convert it to some other substance, like urea or uric acid , which are much less toxic. (wikidoc.org)

When protein are broken down, the nitrogen backbone of the amino acid is cat- catabolized to produce ammonia and produce hyperammonemia. (carbaglu.com)
The Haber process to produce ammonia from the nitrogen in the air was developed by Fritz Haber and Carl Bosch in 1909 and patented in 1910. (wikidoc.org)
Dozens of chemical plants worldwide produce ammonia. (wikidoc.org)

Ammonia incorporation in animals occurs through the actions of glutamate dehydrogenase and glutamine synthase. (medmuv.com)
A pair of principal enzymes, glutamate dehydrogenase and glutamine synthatase, are found in all organisms and effect the conversion of ammonia into the amino acids glutamate and glutamine, respectively. (medmuv.com)

Deamination of amino acids is the major source of intracellular ammonia and the reaction process has two main steps: the first step is the generation of glutamate or glutamine mediated by transamination, and the second step is the deamination of glutamate or glutamine. (csi.org.cn)

[1] Although ammonia is regulated in the United States as a non-flammable gas, it still meets the definition of a material that is toxic by inhalation and requires a hazardous safety permit when transported in quantities greater than 13,248 L (3,500 gallons). (wikidoc.org)
Ammonia the highly toxic product of protein catabolism, is rapidly inactivated by a variety of reactions. (medmuv.com)
It is the primary vehicle for the removal of toxic ammonia from the body. (biotrend.com)

A second molecule of ATP then phosphorylates carbamic acid, creating carbamoyl phosphate. (wikipedia.org)
The ammonia molecule has a trigonal pyramid shape, as predicted by VSEPR theory . (wikidoc.org)
The nitrogen atom in the molecule has a lone electron pair , and ammonia acts as a base , a proton acceptor. (wikidoc.org)
This shape gives the molecule an overall dipole moment and makes it polar so that ammonia readily dissolves in water . (wikidoc.org)
Nevertheless, the catabolism of proteins releases the chronic cytotoxic molecule ammonia (NH 3 ). (csi.org.cn)

The degree to which ammonia forms the ammonium ion increases upon lowering the pH of the solution - at "physiological" pH (~7), about 99% of the ammonia molecules are protonated . (wikidoc.org)

In addition, patients were advised to control their dietary intake of proteins, which are rich in nitrogen, to reduce the amount of ammonia formed in the body. (europa.eu)
This allows the levels of nitrogen in the body to decrease, reducing the amount of ammonia produced. (europa.eu)
Reduced nitrogen enters the human body as dietary free amino acids, protein, and the ammonia produced by intestinal tract bacteria. (medmuv.com)
and R.sup.2 and R.sup.3 are each independently hydrogen or carbamoyl or taken together are a nitrogen and carbon containing heterocyclic ring. (justia.com)

In the liver, alanine transaminase transfers the ammonia to α-KG and regenerates pyruvate. (medmuv.com)

But anytime that there is a triggering event, such as an infection or increased intake of protein or sometime just plain stress or the administration of steroid, patient can become symptomatic with an elevated ammonia level. (carbaglu.com)
Pyridoxal phosphate biosynthetic protein PdxA [Interproscan]. (ntu.edu.sg)

MGS-like domain, Carbamoyl-phosphate synthase L chain, Carbamoyl-phosphate synthetase large chain [Interproscan]. (ntu.edu.sg)

These inter-domain tunnels connect the three distinct active sites, and function as conduits for the transport of unstable reaction intermediates (ammonia and carbamate) between successive active sites. (wikipedia.org)

Carboxy phosphate reacts with ammonia to give carbamic acid. (wikipedia.org)
In turn, carbamic acid reacts with a second ATP to give carbamoyl phosphate plus ADP. (wikipedia.org)
The carboxylphosphate then reacts with ammonia to form carbamic acid, releasing inorganic phosphate. (wikipedia.org)
At a later period, when sal-ammoniac was obtained by distilling the hoofs and horns of oxen and neutralizing the resulting carbonate with hydrochloric acid , the name "spirit of hartshorn" was applied to ammonia. (wikidoc.org)
The cycle converts two amino groups, one from NH 4 + and one from Aspartic acid , and a carbon atom from HCO 3 - , to relatively nontoxic excretion product, urea , at the cost of four "high-energy" phosphate bonds (3 ATP hydrolyzed to 2 ADP and one AMP). (wikidoc.org)

Here we show that CD8+ memory T (TM) cells mobilize the carbamoyl phosphate (CP) metabolic pathway to clear ammonia, thus promoting memory development. (biomed.news)

Because of its many uses, ammonia is one of the most highly produced inorganic chemicals. (wikidoc.org)

The urea cycle (also known as the ornithine cycle ) is a cycle of biochemical reactions occurring in many animals that produces urea from ammonia (NH 3 ). (wikidoc.org)

It is a key intermediate in the urea cycle, the pathway by which mammals excrete ammonia. (diff.org)

Ammonia is a compound with the formula N H 3 . (wikidoc.org)

however, the N-terminal carboxy phosphate domain catalyses the phosphorylation of biocarbonate, while the C-terminal domain catalyses the phosphorylation of the carbamate intermediate. (wikipedia.org)

The cells of various tissues and organs have a powerful antioxidant (free radical scavenging) function, which has been carried out extensive research, but the study of ammonia clearance is very limited except for the liver cells convert NH 3 to urea for detoxification. (csi.org.cn)

In the form of sal-ammoniac, ammonia was known to the alchemists as early as the 13th century, being mentioned by Albertus Magnus. (wikidoc.org)

Ammonia is found in small quantities in the atmosphere, being produced from the putrefaction of nitrogenous animal and vegetable matter. (wikidoc.org)

The strength of such solutions is measured in units of baume (density), with 26 degrees baume (about 30 weight percent ammonia at 15.5 °C) being the typical high concentration commercial product. (wikidoc.org)
[3] Household ammonia ranges in concentration from 5 to 10 weight percent ammonia. (wikidoc.org)

Anatomy2

Organisms6

Chemicals and Drugs86

Analytical, Diagnostic and Therapeutic Techniques and Equipment4

Phenomena and Processes12

Information Science3

Carbamyl phosphate2

Synthesis6

Enzyme7

Citrulline6

Cytosol2

Bicarbonate2

Small subunit1

Metabolism3

NAGS2

Subunit3

Ornithine transcarbamylase1

Ensembl1

UCDs1

Accumulation2

Deficiency2

Inhibition1

Escherichia1

Plasma ammonia2

Remove ammonia1

Produce ammonia3

Glutamate Dehydrogenase2

Glutamine1

Toxic3

Molecule5

Ammonium ion1

Nitrogen4

Pyruvate1

Protein2

Interproscan1

Reaction1

Acid5

Metabolic1

Inorganic1

Biochemical1

Pathway1

Compound1

Intermediate1

Liver1

Form1

Nitrogenous1

Concentration2