and the synthesis of pyrimidines. Its enzymatic counterpart, carbamoyl phosphate synthetase I (CPS I), interacts with a class of molecules called sirtuins...

Carbamoyl phosphate synthetase I deficiency (CPS I deficiency) is an autosomal recessive metabolic disorder that causes ammonia to accumulate in the blood...

Carbamoyl phosphate synthetase I (CPS I) is a ligase enzyme located in the mitochondria involved in the production of urea. Carbamoyl phosphate synthetase...

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...

Carbamoyl phosphate synthetase III (CPS III) is one of the three isoforms of the carbamoyl phosphate synthetase, an enzyme that catalyzes the active production...

Carbamoyl phosphate synthetase (glutamine-hydrolysing) (EC 6.3.5.5) is an enzyme that catalyzes the reactions that produce carbamoyl phosphate in the cytosol...

carbamoyl phosphate synthetase I and requires the use of two ATP molecules. The carbamoyl phosphate then enters the urea cycle. Carbamoyl phosphate is...

of an enzyme with this activity is carbamoyl phosphate synthetase, e.g. carbamoyl phosphate synthetase I carrying out the first step of the urea cycle...

the condensation of L-aspartate and carbamoyl phosphate to form N-carbamoyl-L-aspartate and inorganic phosphate. The catalysis by ATCase serves as the...

process are all coded by the same gene in CAD which consists of carbamoyl phosphate synthetase II, aspartate carbamoyltransferase and dihydroorotase. Dihydroorotate...

CAD protein (carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase) is a trifunctional multi-domain enzyme involved in the first...

Ornithine translocase deficiency, also called hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome, is a rare autosomal recessive urea cycle...

into carbamoyl phosphate (H 2NC(O)OPO2− 3) by carbamoyl phosphate synthetase. Ornithine transcarbamylase then catalyzes the reaction between carbamoyl phosphate...

chili peppers Carbamoyl phosphate synthase II, an enzyme that catalyzes a reaction yielding carbamoyl phosphate Carbamoyl phosphate synthetase, an enzyme...

converts carbamoyl aspartic acid into 4,5-dihydroorotic acid in the biosynthesis of pyrimidines. It forms a multifunctional enzyme with carbamoyl phosphate synthetase...

(OMIM): 237300 - hyperammonemia due to carbamoyl phosphate synthetase I deficiency (carbamoyl phosphate synthetase I) Online Mendelian Inheritance in Man...

carbamoyl phosphate synthetase I, a mitochondrial enzyme that catalyzes the first and rate-limiting step of the urea cycle by synthesizing carbamoyl phosphate...

example, RHEB is an activator for nucleotide synthesis by binding carbamoyl-phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase (CAD), an...

negligible, as is the case with N-acetylglutamate's activity on carbamoyl phosphate synthetase I, for example. A non-regulatory allosteric site is any non-regulatory...

in the bloodstream. Ammonia is converted to carbamoyl phosphate by the enzyme carbamoyl phosphate synthetase, and then enters the urea cycle to be either...

converted into carbamoyl phosphate through the investment of two ATP molecules. This step is facilitated by carbamoyl phosphate synthetase I. The second...

"Required allosteric effector site for N-acetylglutamate on carbamoyl-phosphate synthetase I". The Journal of Biological Chemistry. 271 (30): 18285–94...

to form glutamine: Glutamate + ATP + NH3 → Glutamine + ADP + phosphate Glutamine synthetase uses ammonia produced by nitrate reduction, amino acid degradation...

is to plants, because opisthokonts have a triple fusion of carbamoyl phosphate synthetase, dihydroorotase, and aspartate carbamoyltransferase that is...

and phosphates. Uracil serves as allosteric regulator and coenzyme for reactions in animals and in plants. UMP controls the activity of carbamoyl phosphate...

homologous to other N-terminal nucleophile (Ntn) hydrolases such as carbamoyl phosphate synthetase (CPSase). Nine invariant residues among the sequences of all...

author of an NEJM paper in which a child with a liver disorder, Carbamoyl phosphate synthetase I deficiency, received CRISPR gene editing technology. The disorder...

glutaric acidemia type 1 Urea Cycle Disorder or Urea Cycle Defects Carbamoyl phosphate synthetase I deficiency Citrullinemia type II (citrin deficiency) Disorders...

deficiences in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic acid synthetase. Uncontrolled, this causes...

used for the treatment of hyperammonaemia. Carglumic acid is a carbamoyl phosphate synthetase 1 (CPS 1) activator. The most common adverse effects include...