Search results for "RESPIRATORY-CHAIN"

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A spontaneous mitonuclear epistasis converging on Rieske Fe-S protein exacerbates complex III deficiency in mice

2020

We previously observed an unexpected fivefold (35 vs. 200 days) difference in the survival of respiratory chain complex III (CIII) deficient Bcs1lp.S78G mice between two congenic backgrounds. Here, we identify a spontaneous homoplasmic mtDNA variant (m.G14904A, mt-Cybp.D254N), affecting the CIII subunit cytochrome b (MT-CYB), in the background with short survival. We utilize maternal inheritance of mtDNA to confirm this as the causative variant and show that it further decreases the low CIII activity in Bcs1lp.S78G tissues to below survival threshold by 35 days of age. Molecular dynamics simulations predict D254N to restrict the flexibility of MT-CYB ef loop, potentially affecting RISP dyna…

DYNAMICSepistasisMale0301 basic medicineNon-Mendelian inheritanceMitochondrial DiseasesMetabolic disordersRespiratory chainGeneral Physics and AstronomyDISEASEmitokondriotauditElectron Transport Complex IIIMice0302 clinical medicineenergy metabolismCRYSTAL-STRUCTUREIRON-SULFUR PROTEINlcsh:ScienceMice KnockoutGeneticsmitokondrio-DNAMultidisciplinaryCYTOCHROME BC(1) COMPLEXCytochrome bQCytochromes bMitochondria3. Good healthFemaleRESPIRATORY-CHAINGRACILE SYNDROMEhenkiinjääminenOxidation-ReductionMitochondrial DNAMitochondrial diseaseScienceCongenicMolecular Dynamics SimulationBiologyDNA MitochondrialArticleGeneral Biochemistry Genetics and Molecular Biology03 medical and health sciencesaineenvaihduntahäiriötmedicinemetabolic disordersAnimalsMUTATIONSEpistasis GeneticEnergy metabolismGeneral ChemistryCytochrome b Groupmedicine.diseaseMice Inbred C57BL030104 developmental biologyCoenzyme Q – cytochrome c reductaseEpistasis1182 Biochemistry cell and molecular biologyATPases Associated with Diverse Cellular ActivitiesEpistasislcsh:QGUI MEMBRANE-BUILDERkoe-eläinmallitMetabolism Inborn Errors030217 neurology & neurosurgeryGENERATIONMolecular ChaperonesNature Communications
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The desert gerbil Psammomys obesus as a model for metformin-sensitive nutritional type 2 diabetes to protect hepatocellular metabolic damage: Impact …

2017

Introduction While metformin (MET) is the most widely prescribed antidiabetic drug worldwide, its beneficial effects in Psammomys obesus (P. obesus), a rodent model that mimics most of the metabolic features of human diabetes, have not been explored thoroughly. Here, we sought to investigate whether MET might improve insulin sensitivity, glucose homeostasis, lipid profile as well as cellular redox and energy balance in P. obesus maintained on a high energy diet (HED). Materials and methods P. obesus gerbils were randomly assigned to receive either a natural diet (ND) consisting of halophytic plants (control group) or a HED (diabetic group) for a period of 24 weeks. MET (50 mg/kg per os) was…

Male0301 basic medicinePhysiologymedicine.medical_treatment[SDV]Life Sciences [q-bio]Body-WeightRespiratory chainlcsh:MedicineMitochondria LiverBiochemistrychemistry.chemical_compoundLiver Parenchymal-CellsEndocrinologyGlucose MetabolismAnimal CellsKetogenesisMedicine and Health SciencesElectrochemistryGlucose homeostasisGut Microbiotalcsh:ScienceEnergy-Producing OrganellesComputingMilieux_MISCELLANEOUS2. Zero hungerMultidisciplinaryOrganic CompoundsMonosaccharidesFatty AcidsChemical ReactionsLipidsMetforminMitochondria3. Good healthChemistryPhysiological ParametersLiverPhysical SciencesCarbohydrate MetabolismCellular Structures and OrganellesCellular TypesAnatomyOxidation-ReductionResearch Articlemedicine.medical_specialtyIsolated Rat HepatocytesEndocrine DisordersCarbohydratesBioenergeticsBiologyCarbohydrate metabolism03 medical and health sciencesInsulin resistanceInternal medicineFood-IntakeDiabetes MellitusmedicineAnimalsHypoglycemic AgentsObesityRespiratory-Chain[ SDV ] Life Sciences [q-bio]Fatty acid metabolismInsulinBody WeightOrganic Chemistrylcsh:RChemical CompoundsGluconeogenesisBiology and Life SciencesCell Biologymedicine.diseaseGlucose-6-Phosphate HydrolysisDisease Models AnimalGlucoseMetabolism030104 developmental biologyEndocrinologyDiabetes Mellitus Type 2GluconeogenesischemistryMetabolic DisordersHepatocyteslcsh:QInsulin ResistanceGerbillinaeGlucose-ProductionFatty-Acid-MetabolismOxidation-Reduction Reactions
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