Search results for "urea cycle"

showing 10 items of 17 documents

Urea cycle dysregulation in non-alcoholic fatty liver disease.

2018

Background & Aims: In non-alcoholic steatohepatitis (NASH), the function of urea cycle enzymes (UCEs) may be affected, resulting in hyperammonemia and the risk of disease progression. We aimed to determine whether the expression and function of UCEs are altered in an animal model of NASH and in patients with non-alcoholic fatty liver disease (NAFLD), and whether this process is reversible. Methods: Rats were first fed a high-fat, high-cholesterol diet for 10 months to induce NASH, before being switched onto a normal chow diet to recover. In humans, we obtained liver biopsies from 20 patients with steatosis and 15 with NASH. Primary rat hepatocytes were isolated and cultured with free fatty …

AdultMale0301 basic medicinemedicine.medical_specialtyCarbamoyl-Phosphate Synthase (Ammonia)Ornithine transcarbamylase03 medical and health scienceschemistry.chemical_compound0302 clinical medicineAmmoniaGlutamate-Ammonia LigaseNon-alcoholic Fatty Liver DiseaseInternal medicineGene expressionmedicineAnimalsHumansUreaRats WistarPromoter Regions GeneticCells CulturedOrnithine CarbamoyltransferaseAgedHepatologyChemistryFatty liverHyperammonemiaDNA MethylationMiddle Agedmedicine.diseaseRats030104 developmental biologyEndocrinologyLiverUrea cycleHepatocytesUreaFemale030211 gastroenterology & hepatologySteatohepatitisSteatosis
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Functional characterization of hepatocytes for cell transplantation: customized cell preparation for each receptor.

2009

The first indication of hepatocyte transplantation is inborn liver-based metabolic disorders. Among these, urea cycle disorders leading to the impairment to detoxify ammonia and Crigler-Najjar Syndrome type I, a deficiency in the hepatic UDP-glucuronosyltransferase 1A1 present the highest incidence. Metabolically qualified human hepatocytes are required for clinical infusion. We proposed fast and sensitive procedures to determine their suitability for transplantation. For this purpose, viability, attachment efficiency, and metabolic functionality (ureogenic capability, cytochrome P450, and phase II activities) are assayed prior to clinical cell infusion to determine the quality of hepatocyt…

AdultMaleAdolescentCell SurvivalCell TransplantationCellBiomedical Engineeringlcsh:MedicineReceptors Cell SurfaceCell SeparationPharmacologyCold Ischemia TimeDonor Selectionchemistry.chemical_compoundYoung AdultmedicineHumansUreaGlucuronosyltransferaseReceptorChildUrea Cycle Disorders InbornCells CulturedAgedCrigler-Najjar SyndromeAged 80 and overTransplantationLiver DiseasesMetabolic disorderlcsh:RCold IschemiaGraft SurvivalInfant NewbornInfantCell BiologyMiddle Agedmedicine.diseaseTransplantationmedicine.anatomical_structurechemistryUrea cycleChild PreschoolUreaHepatocytesBiological AssayFemaleSteatosisCell transplantation
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Urea Cycle Metabolites and Atrial Fibrillation or Heart Failure Risk: Two Case-Control Studies in the PREDIMED Trial

2021

OBJECTIVES: To prospectively analyze the associations between urea cycle metabolites and incident atrial fibrillation (AF) or heart failure (HF), and to evaluate the effect of a Mediterranean diet (MD) intervention on such associations. METHODS: We designed two nested case-control studies within the PREDIMED trial, a randomized controlled trial aimed to evaluate the effect of two MD interventions, supplemented with either extra virgin olive oil (EVOO) or nuts, on cardiovascular disease (CVD). Fasting blood samples were collected at baseline and urea cycle metabolites (arginine, citrulline, and ornithine) and methylarginines (asymmetric dimethylarginine/symmetric dimethylarginine ratio (ADMA…

Aging and Chronic DiseaseNutrition and DieteticsArginineMediterranean dietDiet therapybusiness.industryMedicine (miscellaneous)Atrial fibrillationPharmacologyOrnithinemedicine.diseasechemistry.chemical_compoundchemistryHeart failureUrea cyclemedicineCitrullinebusinessFood ScienceCurrent Developments in Nutrition
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Key Enzymes in Pyrimidine Synthesis, CAD and CPS1, Predict Prognosis in Hepatocellular Carcinoma

2021

Simple Summary Individual patients with liver cancer have a highly variable clinical course. Hence, there is an urgent need to identify new prognostic markers to determine prognosis and select specific therapies. Expression of two key enzymes in pyrimidine synthesis was analyzed in a large, well-characterized cohort of patients with liver cancer. Dysregulated expression of these enzymes was associated with shorter survival of the patients. A combined score of both markers was found to be a statistically independent prognostic marker. Abstract Patients with hepatocellular carcinoma (HCC) have a highly variable clinical course. Therefore, there is an urgent need to identify new prognostic mar…

Cancer ResearchpyrimidineCADlcsh:RC254-282Article03 medical and health sciences0302 clinical medicinecps1medicineHCCchemistry.chemical_classificationTissue microarraybusiness.industryCancerhepatocellular carcinomaHCCSmedicine.diseaselcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogensurea cycle dysregulationEnzymeDihydroorotasechemistryOncology030220 oncology & carcinogenesisHepatocellular carcinomaPyrimidine metabolismKey (cryptography)Cancer researchImmunohistochemistryBiomarker (medicine)biomarkercad030211 gastroenterology & hepatologyprognosisbusinessCancers
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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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Adaptive physiological water conservation explains hypertension and muscle catabolism in experimental chronic renal failure

2021

Abstract Aim We have reported earlier that a high salt intake triggered an aestivation‐like natriuretic‐ureotelic body water conservation response that lowered muscle mass and increased blood pressure. Here, we tested the hypothesis that a similar adaptive water conservation response occurs in experimental chronic renal failure. Methods In four subsequent experiments in Sprague Dawley rats, we used surgical 5/6 renal mass reduction (5/6 Nx) to induce chronic renal failure. We studied solute and water excretion in 24‐hour metabolic cage experiments, chronic blood pressure by radiotelemetry, chronic metabolic adjustment in liver and skeletal muscle by metabolomics and selected enzyme activity…

Male0301 basic medicinePhysiologyBody waterBlood Pressure030204 cardiovascular system & hematologyRats Sprague-Dawley0302 clinical medicineRegular Paperdouble‐barrier conceptmuscle mass losstransaminationKidneyglycine methylationMusclesurine concentrationglucose‐alanine‐shuttlepurine metabolismaestivationmedicine.anatomical_structuremedicine.drugbody watermedicine.medical_specialtykidneyskinhypertensionorganic osmolytesliverCardivascular PhysiologyNorepinephrine (medication)03 medical and health sciencesCopeptinhepato‐renalInternal medicinemedicineurea cycleAnimalsHumansbody sodiumSalt intakeMuscle SkeletalTransepidermal water lossConservation of Water Resourcesbusiness.industrySkeletal muscletransepidermal water lossWaterdehydrationRats030104 developmental biologyBlood pressureEndocrinologyCardiovascular and Metabolic DiseasesKidney Failure ChronicbusinessActa Physiologica (Oxford, England)
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Improved nitrogen metabolism in rats fed on lipid-rich liquid diets

1994

N metabolism was studied in young rats fed on lipid-rich, isonitrogenous, purified liquid diets, a convenient and easy technique for inducing voluntary overfeeding of energy and lipids under controlled nutritional conditions. Overfed rats showed a marked N retention at the expense of a reduced production of urea. The capacities of isolated hepatocytes to synthesize urea and glucose from added precursors were greatly diminished. The activities of the urea cycle enzymes and several enzymes involved in the availability of NH3, for this pathway were concomitantly reduced in overfed animals. Therefore, our results showed an improved N metabolism in overfed rats promoted by the overfeeding of lip…

MaleLiquid dietNitrogenCarbamoyl-Phosphate Synthase (Ammonia)Medicine (miscellaneous)HyperphagiaBiologychemistry.chemical_compoundGlutamate DehydrogenaseAnimalsUreaAmino AcidsRats WistarNitrogen cycleCells Culturedchemistry.chemical_classificationNutrition and DieteticsCatabolismAlanine TransaminaseMetabolismLipidsDietRatsAmino acidGlucoseEnzymeLiverchemistryBiochemistryUrea cycleUreaBritish Journal of Nutrition
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Liver pathology in transient neonatal hyperammonemia.

1983

Ultrastructural investigations have been performed on two cases of transient neonatal hyperammonaemia (TNH). This newly recognized metabolic disorder is chiefly characterized by severe hyperammonaemia in the postnatal period, a comatous state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and, usually, complete recovery. The aetiology is presently unknown. Electron microscopy uncovered rather congruent alterations of hepatocyte structure, with a wide spectrum of mitochondrial lesions, an increase of autophagous bodies with organelle remnants, and changes in the excretory apparatus. Thus, in contrast to some of the hereditary disorders of the urea cycle, no spec…

MalePathologymedicine.medical_specialtyHistologyMitochondria LiverBiologyMitochondrionOrganic aciduriaUltrastructural PathologyPathology and Forensic MedicineAmmoniaInternal medicinemedicineHumansMolecular BiologyStaining and LabelingMetabolic disorderHepatobiliary diseaseInfant NewbornHyperammonemiaCell BiologyGeneral Medicinemedicine.diseaseMicroscopy Electronmedicine.anatomical_structureEndocrinologyLiverUrea cycleHepatocyteAnatomyMetabolism Inborn ErrorsVirchows Archiv. A, Pathological anatomy and histopathology
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Effect of Thyroid Hormones on Urea Biosynthesis and Related Processes in Rat Liver*

1988

The results of the few studies on the effect of the thyroid status on nitrogen metabolism have been inconclusive and/or contradictory. In an attempt to elucidate this important relationship, we have studied the effect of experimental hypo- and hyperthyroidism on urea biosynthesis and related processes. We have found that the capacity of the liver to synthesize urea was increased in hypothyroid rats, as were the activities of the urea cycle enzymes; there were also changes in the activities of some related enzymes and in the levels of intermediates and amino acids. Isolated hepatocytes from these rats showed an increased capacity for urea synthesis. In hyperthyroid rats the picture was more …

MaleThyroid Hormonesendocrine systemmedicine.medical_specialtyendocrine system diseasesCarbamoyl-Phosphate Synthase (Ammonia)HyperthyroidismIodide PeroxidaseGlucagonchemistry.chemical_compoundEndocrinologyGlutamatesHypothyroidismBiosynthesisAmmoniaInternal medicineCyclic AMPmedicineAnimalsUreaAmino AcidsOrnithine Carbamoyltransferasechemistry.chemical_classificationCatabolismRats Inbred StrainsMetabolismGlucagonRatsAmino acidThyroxineEndocrinologymedicine.anatomical_structureLiverchemistryBiochemistryUrea cycleHepatocyteUreaTriiodothyroninehormones hormone substitutes and hormone antagonistsEndocrinology
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Vitamin A Deficiency Increases Protein Catabolism and Induces Urea Cycle Enzymes in Rats

2010

Chronic vitamin A deficiency induces a substantial delay in the rates of weight and height gain in both humans and experimental animals. This effect has been associated with an impaired nutrient metabolism and loss of body protein. Therefore, we analyzed the effect of vitamin A deficiency on endogenous proteolysis and nitrogen metabolism and its reversibility with all-trans retinoic acid (RA). Male weanling rats, housed in pairs, were pair-fed a vitamin A-deficient (VAD) or control diet until they were 60 d old. A group of deficient rats were further treated with daily intraperitoneal injections of all-trans RA for 10 d. Final body and tissue (i.e. liver and heart) weights were significantl…

MaleVitaminmedicine.medical_specialtyNitrogenMedicine (miscellaneous)TretinoinBiologyAntioxidantsRetinoidschemistry.chemical_compoundInternal medicinemedicineAnimalsUreaMuscle SkeletalTriglyceridesNutrition and DieteticsVitamin A DeficiencyCatabolismRetinolProtein turnoverMethylhistidinesmedicine.diseaseRatsVitamin A deficiencyProtein catabolismEndocrinologyLiverchemistryEnzyme InductionUrea cycleLipid PeroxidationEnergy sourceThe Journal of Nutrition
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