Search results for "Carbamoyl phosphate synthetase"

showing 3 items of 3 documents

The Study of Carbamoyl Phosphate Synthetase 1 Deficiency Sheds Light on the Mechanism for Switching On/Off the Urea Cycle

2015

12 páginas, 4 figuras, 2 tablas.

Conformational changeCarbamoyl-Phosphate Synthase I Deficiency DiseaseAllosteric regulationCarbamoyl-Phosphate Synthase (Ammonia)Urea cycle diseases610 Medicine & healthBiologyMolecular Dynamics Simulationurologic and male genital diseases03 medical and health sciences0302 clinical medicineGlutamates1311 GeneticsAmmoniaEnzyme StabilityGeneticsmedicine1312 Molecular BiologyHumansUreaHyperammonemiaSite-directed mutagenesisMolecular Biology030304 developmental biologychemistry.chemical_classification0303 health sciencesSite-directed mutagenesisurogenital systemMutagenesisCarbamoyl phosphate synthetase 1HyperammonemiaCarbamoyl phosphate synthetasemedicine.diseaseAllosteric regulation3. Good healthProtein Structure TertiaryRestrained molecular dynamicsKineticsEnzymeBiochemistrychemistry10036 Medical ClinicEnzymeUrea cycleMutationInborn errors030217 neurology & neurosurgerySignal Transduction
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N-acetyl-L-glutamate in brain: assay, levels, and regional and subcellular distribution.

1991

N-Acetyl-L-glutamate (NAG), the activator of mitochondrial carbamoyl phosphate synthetase (CPS), is demonstrated by several methods, including a new HPLC assay, in the brain of mammals and of chicken. The brain levels of NAG are 200-300 times lower than the levels of N-acetyl-L-aspartate (NAA), and are similar to the levels of NAG in rat liver. The NAG levels in chicken liver are very low. Although NAG is mitochondrial in the liver, it is cytosolic in brain. Using enzyme activity and immuno assays we did not detect CPS in brain (detection limit, 12.5 micrograms/g brain), excluding that brain NAG is involved in citrullinogenesis. The regional distribution of brain NAG differs from that of NA…

MaleCentral nervous systemurologic and male genital diseasesBiochemistryCellular and Molecular NeuroscienceMiceGlutamatesSpecies SpecificitymedicineAnimalsChromatography High Pressure Liquidchemistry.chemical_classificationN acetyl L glutamateBrain ChemistryAspartic AcidSheepbiologyurogenital systemActivator (genetics)Rats Inbred StrainsGeneral MedicineCarbamoyl phosphate synthetaseEnzyme assayRatsCytosolSubcellular distributionEnzymemedicine.anatomical_structurechemistryBiochemistrybiology.proteinChickensNeurochemical research
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Affinity Cleavage of Carbamoyl-Phosphate Synthetase I Localizes Regions of the Enzyme Interacting with the Molecule of ATP that Phosphorylates Carbam…

1995

Two ATP molecules are used in the reaction catalyzed by carbamoyl-phosphate synthetase I. One molecule (ATPA) phosphorylates HCO3- and the other (ATPB) phosphorylates carbamate. Carbamoyl-phosphate synthetase I is a 160-kDa polypeptide consisting of a 40-kDa N-terminal moiety and a 120-kDa C-terminal moiety, the latter being composed of two similar halves of molecular mass 60 kDa. We showed [Alonso, E., Cervera, J., Garcia-Espana, A., Bendala, E. & Rubio, V. (1992) J. Biol. Chem. 267, 4524-4532] that Fe.ATP bound at the site for ATPB catalyzes the oxidative inactivation of carbamoyl-phosphate synthetase I in a model oxidative system consisting of Fe3+, ascorbate, and O2, and we detected ATP…

chemistry.chemical_classificationbiologyChemistryProtein domainCarbamate kinaseCleavage (embryo)BiochemistryCarbamoyl phosphate synthetase IAmino acidBiochemistrybiology.proteinMoietyNucleotideBinding siteEuropean Journal of Biochemistry
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