0000000000076769

AUTHOR

José A. Botella

showing 3 related works from this author

Altered lipid metabolism in a Drosophila model of Friedreich's ataxia

2010

13 páginas, 5 figuras.-- et al.

MaleAtaxiaCell SurvivalLipid Metabolism Disordersmedicine.disease_causeNervous SystemAnimals Genetically ModifiedLipid peroxidationchemistry.chemical_compoundDownregulation and upregulationIron-Binding ProteinsLipid dropletGeneticsmedicineAnimalsDrosophila ProteinsHumansMolecular BiologyGenetics (clinical)Membrane GlycoproteinsbiologyCélulas glialesFatty AcidsLipid metabolismArticlesGeneral MedicineCell biologyDisease Models AnimalOxidative Stressmedicine.anatomical_structurechemistryBiochemistryFriedreich AtaxiaFrataxinbiology.proteinNeurogliaDrosophilaLipid Peroxidationmedicine.symptomCarrier ProteinsNeurogliaOxidative stress
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Overexpression of Human and Fly Frataxins in Drosophila Provokes Deleterious Effects at Biochemical, Physiological and Developmental Levels

2011

10 pages, 5 figures. 21779322[PubMed] PMCID: PMC3136927

Transgeneved/biology.organism_classification_rank.speciesBlotting WesternLongevitylcsh:MedicineMitochondrionMotor ActivityAconitaseAnimals Genetically ModifiedModel OrganismsIron-Binding ProteinsMorphogenesisGeneticsAnimalsHumansModel organismlcsh:ScienceBiologyGeneticsAconitate HydrataseGene knockdownBrain DiseasesMultidisciplinaryMovement Disordersbiologyved/biologyDrosophila Melanogasterfungilcsh:RAnimal Modelsbiology.organism_classificationPhenotypeImmunohistochemistryMitochondriaOxidative StressNeurologyFriedreich AtaxiaGenetics of DiseaseFrataxinbiology.proteinChromatography GelMedicinelcsh:QDrosophilaDrosophila melanogasterResearch ArticleDevelopmental BiologyPLoS ONE
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Causative role of oxidative stress in a Drosophila model of Friedreich ataxia

2006

Friedreich ataxia (FA), the most common form of hereditary ataxia, is caused by a deficit in the mitochondrial protein frataxin. While several hypotheses have been suggested, frataxin function is not well understood. Oxidative stress has been suggested to play a role in the pathophysiology of FA, but this view has been recently questioned, and its link to frataxin is unclear. Here, we report the use of RNA interference (RNAi) to suppress the Drosophila frataxin gene (fh) expression. This model system parallels the situation in FA patients, namely a moderate systemic reduction of frataxin levels compatible with normal embryonic development. Under these conditions, fh-RNAi flies showed a shor…

AtaxiaBlotting WesternLongevityGene ExpressionCHO Cellsmedicine.disease_causeBiochemistryAconitaseMitochondrial ProteinsCricetulusRNA interferenceCricetinaeIron-Binding ProteinsGeneticsmedicineAnimalsDrosophila ProteinsRNA MessengerMolecular BiologyGeneAconitate HydrataseHyperoxiaGeneticsElectron Transport Complex IbiologyReverse Transcriptase Polymerase Chain ReactionSuccinate dehydrogenasefungiImmunohistochemistryCell biologySuccinate DehydrogenaseOxidative StressDrosophila melanogasterFriedreich AtaxiaFrataxinbiology.proteinRNA Interferencemedicine.symptomOxidative stressBiotechnologyThe FASEB Journal
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