Carbamoyl phosphate synthetase I information

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

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

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

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...

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

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...

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

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

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...

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...

cycle disorders, citrullinemia type I needs to be distinguished from the others: carbamoyl phosphate synthetase deficiency, argininosuccinic acid lyase...

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

Capillary malformation-arteriovenous malformation; 608354; RASA1 Carbamoyl phosphate synthetase I deficiency; 237300; CPS1 Carbohydrate-deficient glycoprotein...

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

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

N-Carbamoylaspartate by undergoing a condensation reaction with carbamoyl phosphate. Dihydroorotase and dihydroorotase dehydrogenase then converts N-Carbamoylaspartate...

a transfer is written as carbamoyl phosphate + L-aspartate → {\displaystyle \rightarrow } L-carbamoyl aspartate + phosphate. Enzymes that transfer aldehyde...

of carbamoyl phosphate from glutamine and CO2. Next, aspartate carbamoyltransferase catalyzes a condensation reaction between aspartate and carbamoyl phosphate...

lysine residues of proteins. SIRT5 deacetylases and regulates carbamoyl phosphate synthetase (CPS1), the rate-limiting and initiating step of the urea cycle...

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

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

(ADC) synthase; CTP synthase; GMP synthase; glutamine-dependent carbamoyl-phosphate synthase; phosphoribosylformylglycinamidine synthase II; and the...

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