Carbamoyl phosphate synthetase
"Inhibition of carbamoyl-phosphate synthase (ammonia) by Tris and Hepes. Effect on Ka for N-acetylglutamate". The Biochemical ... Carbamoyl phosphate synthetase III (found in fish). Carbamoyl phosphate synthase has three main steps in its mechanism and is, ... Carbamoyl phosphate synthetase catalyzes the ATP-dependent synthesis of carbamoyl phosphate from glutamine (EC 6.3.5.5) or ... Carboxy phosphate reacts with ammonia to give carbamic acid. In turn, carbamic acid reacts with a second ATP to give carbamoyl ...
Methylmalonyl-CoA mutase deficiency
... such as ammonia-dependent-carbamoyl-phosphate synthase or CPS1), and inhibits its mechanism of action. The combination of ... Ammonia test, blood count, CT scan, MRI scan, electrolyte levels, genetic testing, methylmalonic acid blood test, and blood ...
Carbamoyl phosphate synthase II
"Carbamoyl-phosphate synthetase. Creation of an escape route for ammonia". The Journal of Biological Chemistry. 277 (42): 39722- ... Carbamoyl phosphate synthetase I Carbamoyl phosphate synthetase III Anderson PM, Meister A (December 1965). "Evidence for an ... phosphate + carbamoyl phosphate It is activated by ATP and PRPP and it is inhibited by UTP (Uridine triphosphate) Neither CPSI ... phosphate + L-glutamate + carbamoyl phosphate (overall reaction) (1a) L-glutamine + H2O ⇌ {\displaystyle \rightleftharpoons } L ...
List of MeSH codes (D08)
... argininosuccinate synthase MeSH D08.811.464.259.350 - carbamoyl-phosphate synthase (ammonia) MeSH D08.811.464.259.400 - carbon- ... carbamoyl-phosphate synthase (glutamine-hydrolyzing) MeSH D08.811.464.259.550 - formate-tetrahydrofolate ligase MeSH D08.811. ... amide synthases MeSH D08.811.464.259.200.200 - aspartate-ammonia ligase MeSH D08.811.464.259.200.600 - glutamate-ammonia ligase ... ammonia-lyases MeSH D08.811.520.232.400.200 - aspartate ammonia-lyase MeSH D08.811.520.232.400.350 - ethanolamine ammonia-lyase ...
Mitochondrial matrix
In the first step ammonia is converted into carbamoyl phosphate through the investment of two ATP molecules. This step is ... The energy is used in order to rotate ATP synthase which facilitates the passage of a proton, producing ATP. A pH difference ... The urea cycle makes use of L-ornithine, carbamoyl phosphate, and L-citrulline. The electron transport chain oxidizes coenzymes ... The urea cycle is facilitated by carbamoyl phosphate synthetase I and ornithine transcarbamylase. β-Oxidation uses pyruvate ...
Citrullinemia type I
As one of the urea cycle disorders, citrullinemia type I needs to be distinguished from the others: carbamoyl phosphate ... As ammonia accumulates further, the affected infant may enter a hyperammonemic coma, which indicates neurological damage and ... and N-Acetylglutamate synthase deficiency. Other diseases that may appear similar to CTLN1 include the organic acidemias and ... Signs and symptoms of CTLN1 in infants are caused by increasing levels of ammonia in the blood and cerebrospinal fluid and ...
List of EC numbers (EC 6)
... carbamoyl-phosphate synthase (ammonia) EC 6.3.4.17: formate-dihydrofolate ligase EC 6.3.4.18: 5-(carboxyamino)imidazole ... carbamoyl-phosphate synthase (glutamine-hydrolysing) EC 6.3.5.6: asparaginyl-tRNA synthase (glutamine-hydrolysing) EC 6.3.5.7: ... glutathionylspermidine synthase EC 6.3.1.9: trypanothione synthase EC 6.3.1.10: adenosylcobinamide-phosphate synthase EC 6.3. ... argininosuccinate synthase EC 6.3.4.6: urea carboxylase EC 6.3.4.7: ribose-5-phosphate-ammonia ligase EC 6.3.4.8: ...
Hyperammonemia
... hyperammonemia due to carbamoyl phosphate synthetase I deficiency (carbamoyl phosphate synthetase I) Online Mendelian ... Urease producers form ammonia and carbon dioxide from urea. Ammonia then enters the systemic circulation (most venous supply of ... hyperammonemia due to N-Acetylglutamate synthase deficiency Online Mendelian Inheritance in Man (OMIM): 237300 - ... Arginase deficiency Citrullinemia N-acetylglutamate synthetase deficiency Ornithine translocase deficiency Carbamoyl phosphate ...
Aminoacylase
N-acetyl-L-glutamate is an allosteric activator of carbamoyl phosphate synthetase, a crucial enzyme that commits NH4+ molecules ... The urea cycle gets rid of excess ammonia (NH4+) in the body, a process that must be up-regulated during times of increased ... When amino acid catabolism increases, N-Acetylglutamate synthase is up-regulated, producing more N-acetyl-L-glutamate, which up ... which down-regulates carbamoyl phosphate synthetase and the rest of the urea cycle. This response is evolutionarily ...
Citrulline
... or glutamine/glutamate from asymmetric dimethylarginine via DDAH Citrulline is made from ornithine and carbamoyl phosphate in ... catalyzed by nitric oxide synthase. Citrulline can be derived from: from arginine via nitric oxide synthase, as a byproduct of ... It is a key intermediate in the urea cycle, the pathway by which mammals excrete ammonia by converting it into urea. Citrulline ... "Nos2 - Nitric Oxide Synthase". Uniprot.org. Uniprot Consortium. Retrieved 10 February 2015. Cox M, Lehninger AL, Nelson DR ( ...
Urea cycle
To enter the cycle, ammonia is converted to carbamoyl phosphate. The urea cycle consists of four enzymatic reactions: one ... NAcGlu is an obligate activator of carbamoyl phosphate synthetase. Synthesis of NAcGlu by N-acetylglutamate synthase (NAGS) is ... The carbamoyl phosphate then enters the urea cycle. Carbamoyl phosphate is converted to citrulline. With catalysis by ornithine ... N-Acetylglutamate synthase (NAGS) deficiency Carbamoyl phosphate synthetase (CPS) deficiency Ornithine transcarbamoylase (OTC) ...
N-Acetylglutamate synthase deficiency
... in which carbamoyl phosphate is produced. Carbamoyl phosphate synthase 1, abbreviated as CPS1, is activated by its natural ... synthase or a genetic mutation in the gene coding for the enzyme will lead to urea cycle failure in which ammonia is not ... Carbamoyl phosphate synthase I is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the urea ... N-Acetylglutamate synthase deficiency is an autosomal recessive urea cycle disorder. The symptoms are visible within the first ...
Glutamine amidotransferase
CTP synthase; GMP synthase; glutamine-dependent carbamoyl-phosphate synthase; phosphoribosylformylglycinamidine synthase II; ... In molecular biology, glutamine amidotransferases (GATase) are enzymes which catalyse the removal of the ammonia group from a ... Class-I GATase domains have been found in the following enzymes: the second component of anthranilate synthase and 4-amino-4- ... Nyunoya H, Lusty CJ (August 1984). "Sequence of the small subunit of yeast carbamyl phosphate synthetase and identification of ...
List of EC numbers (EC 2)
... carbamoyl-phosphate synthase (ammonia) EC 2.7.2.6: formate kinase EC 2.7.2.7: butyrate kinase EC 2.7.2.8: acetylglutamate ... sucrose-phosphate synthase EC 2.4.1.15: α,α-trehalose-phosphate synthase (UDP-forming) EC 2.4.1.16: chitin synthase EC 2.4.1.17 ... 2-hydroxy-3-oxoadipate synthase EC 2.2.1.6: acetolactate synthase EC 2.2.1.7: 1-deoxy-D-xylulose-5-phosphate synthase EC 2.2. ... 2-ethylmalate synthase EC 2.3.3.7: 3-ethylmalate synthase EC 2.3.3.8: ATP citrate synthase EC 2.3.3.9: malate synthase EC 2.3. ...
Phytotoxin
... enolpyruvylshikimate 3-phosphate synthase enzyme. Photosystem II Inhibitors reduce the electron flow from water to NADPH2+ ... tabaci that may cause toxic concentrations of ammonia to build up. This buildup of ammonia causes leaf chlorosis. Glycopeptides ... which catalyzes the formation of citrulline from ornithine and carbamoylphosphate in the arginine biosynthetic pathway. ...
Uracil
UMP controls the activity of carbamoyl phosphate synthetase and aspartate transcarbamoylase in plants, while UDP and UTP ... The first reaction is the simplest of the syntheses, by adding water to cytosine to produce uracil and ammonia: C4H5N3O + H2O ... In humans this decarboxylation is achieved by the enzyme UMP synthase. In contrast to the purine nucleotides, the pyrimidine ... When a phosphate attaches to uridine, uridine 5′-monophosphate is produced. Uracil undergoes amide-imidic acid tautomeric ...
Biosynthesis
... the enzyme carbamoyl phosphate synthase combining glutamine with CO2 in an ATP dependent reaction to form carbamoyl phosphate. ... Furthermore, the enzyme glutamine synthetase (GS) is able to transfer ammonia onto glutamate and synthesize glutamine, ... Phosphate addition to UMP is catalyzed by a kinase enzyme. The enzyme CTP synthase catalyzes the next reaction step: the ... Aspartate carbamoyltransferase condenses carbamoyl phosphate with aspartate to form uridosuccinate. Dihydroorotase performs ...
Nucleotide
... aspartate carbamoyltransferase catalyzes a condensation reaction between aspartate and carbamoyl phosphate to form carbamoyl ... First, GTP hydrolysis fuels the addition of aspartate to IMP by adenylosuccinate synthase, substituting the carbonyl oxygen for ... and from ammonia and carbon dioxide. Recently it has been also demonstrated that cellular bicarbonate metabolism can be ... The synthesis of the pyrimidines CTP and UTP occurs in the cytoplasm and starts with the formation of carbamoyl phosphate from ...
Alternative abiogenesis scenarios
Pyrimidines are shown to be synthesized from the reaction between aspartate and carbamoyl phosphate at 60 °C and in the ... He placed lumps of lava over amino acids derived from methane, ammonia and water, sterilized all materials, and baked the lava ... The thermosynthesis hypothesis does not even invoke a pathway: ATP synthase's binding change mechanism resembles a physical ... the reactions produce erythrose 4-phosphate, an amino acid precursor and ribose 5-phosphate, a nucleotide precursor. ...
List of EC numbers (EC 4)
... methyl phosphate synthase EC 4.2.3.154: demethyl-4-deoxygadusol synthase EC 4.2.3.155: 2-epi-valiolone synthase EC 4.2.3.156: ... carbamoyl-serine ammonia-lyase EC 4.3.1.14: 3-aminobutyryl-CoA ammonia-lyase EC 4.3.1.15: diaminopropionate ammonia-lyase EC ... limonene synthase EC 4.1.99.11: benzylsuccinate synthase EC 4.1.99.12: 3,4-dihydroxy-2-butanone-4-phosphate synthase EC 4.1. ... deacetylipecoside synthase EC 4.3.3.5: 4′-demethylrebeccamycin synthase EC 4.3.3.6: pyridoxal 5′-phosphate synthase (glutamine ...