0000000000356973

AUTHOR

Juan Botas

showing 4 related works from this author

Metal homeostasis regulators suppress FRDA phenotypes in a drosophila model of the disease

2016

Friedreich's ataxia (FRDA), the most commonly inherited ataxia in populations of European origin, is a neurodegenerative disorder caused by a decrease in frataxin levels. One of the hallmarks of the disease is the accumulation of iron in several tissues including the brain, and frataxin has been proposed to play a key role in iron homeostasis. We found that the levels of zinc, copper, manganese and aluminum were also increased in a Drosophila model of FRDA, and that copper and zinc chelation improve their impaired motor performance. By means of a candidate genetic screen, we identified that genes implicated in iron, zinc and copper transport and metal detoxification can restore frataxin def…

0301 basic medicinePhysiologyGene Expressionlcsh:MedicineMitochondrionmedicine.disease_causeAntioxidantsIron-Binding ProteinsMedicine and Health SciencesHomeostasislcsh:ScienceGeneticsMultidisciplinarybiologyDrosophila MelanogasterIron-binding proteinsAnimal ModelsPhenotypeMitochondria3. Good healthInsectsDNA-Binding ProteinsChemistryZincPhenotypesPhysical SciencesDrosophilaAnatomymedicine.symptomDrosophila melanogasterResearch ArticleChemical ElementsAtaxiaArthropodaIronResearch and Analysis Methods03 medical and health sciencesModel OrganismsOcular SystemmedicineGeneticsAnimalsHumansGenetikManganeselcsh:ROrganismsBiology and Life SciencesCell Biologybiology.organism_classificationInvertebratesOxidative StressDisease Models Animal030104 developmental biologyFriedreich AtaxiaFrataxinbiology.proteinEyeslcsh:QPhysiological ProcessesCarrier ProteinsHeadCopperOxidative stressAluminumTranscription FactorsGenetic screen
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TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich’s Ataxia

2015

Friedreich's ataxia (FRDA), the most common inherited ataxia in the Caucasian population, is a multisystemic disease caused by a significant decrease in the frataxin level. To identify genes capable of modifying the severity of the symptoms of frataxin depletion, we performed a candidate genetic screen in a Drosophila RNAi-based model of FRDA. We found that genetic reduction in TOR Complex 1 (TORC1) signalling improves the impaired motor performance phenotype of FRDA model flies. Pharmacologic inhibition of TORC1 signalling by rapamycin also restored this phenotype and increased the lifespan and ATP levels. Furthermore, rapamycin reduced the altered levels of malondialdehyde + 4-hydroxyalke…

Malelcsh:MedicineGene Expressionmedicine.disease_causeAntioxidantsAnimals Genetically ModifiedAdenosine Triphosphate0302 clinical medicineRNA interferenceIron-Binding ProteinsMalondialdehydeDrosophila Proteinslcsh:ScienceAconitate HydrataseGenetics0303 health sciencesMultidisciplinaryReverse Transcriptase Polymerase Chain ReactionGlutathione3. Good healthCell biologyDrosophila melanogasterRNA Interferencemedicine.symptomImmunosuppressive AgentsDrosophila ProteinResearch ArticleAtaxiaLongevityMotor ActivityBiologyAconitase03 medical and health sciencesmedicineAnimalsHumans030304 developmental biologySirolimusAldehydesSuperoxide Dismutaselcsh:RAutophagyRepressor ProteinsDisease Models AnimalOxidative StressFriedreich AtaxiaFrataxinbiology.proteinlcsh:Q030217 neurology & neurosurgeryOxidative stressTranscription FactorsGenetic screenPLOS ONE
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Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition) 1

2021

Contains fulltext : 232759.pdf (Publisher’s version ) (Closed access) In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to…

0301 basic medicineProgrammed cell deathSettore BIO/06AutophagosomeAutolysosome[SDV]Life Sciences [q-bio]lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4]Autophagy-Related ProteinsReviewComputational biology[SDV.BC]Life Sciences [q-bio]/Cellular BiologyBiologySettore MED/0403 medical and health sciencesstressChaperone-mediated autophagyddc:570AutophagyLC3AnimalsHumanscancerSettore BIO/10Autophagosome; cancer; flux; LC3; lysosome; macroautophagy; neurodegeneration; phagophore; stress; vacuoleSet (psychology)Molecular Biologyvacuole.phagophore030102 biochemistry & molecular biologyvacuolebusiness.industryInterpretation (philosophy)AutophagyAutophagosomesneurodegenerationCell BiologyfluxMulticellular organismmacroautophagy030104 developmental biologyKnowledge baselysosomeAutophagosome; LC3; cancer; flux; lysosome; macroautophagy; neurodegeneration; phagophore; stress; vacuoleBiological AssayLysosomesbusinessBiomarkers[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
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Autophagy

2021

In 2008, we published the first set of guidelines for standardizing research in autophagy. Since then, this topic has received increasing attention, and many scientists have entered the field. Our knowledge base and relevant new technologies have also been expanding. Thus, it is important to formulate on a regular basis updated guidelines for monitoring autophagy in different organisms. Despite numerous reviews, there continues to be confusion regarding acceptable methods to evaluate autophagy, especially in multicellular eukaryotes. Here, we present a set of guidelines for investigators to select and interpret methods to examine autophagy and related processes, and for reviewers to provide…

macroautophagy;autophagyAutophagosome[SDV]Life Sciences [q-bio]canceLC3 macroautophagyautophagosomeneurodegeneration;[SDV.BC]Life Sciences [q-bio]/Cellular BiologyAutophagy AutophagosomeNOstress vacuolestressautophagic processesstrerfluxLC3cancerguidelinesAutophagosome; cancer; flux; LC3; lysosome; macroautophagy; neurodegeneration; phagophore; stress; vacuoleSettore BIO/06 - Anatomia Comparata E Citologia[SDV.BC] Life Sciences [q-bio]/Cellular BiologyComputingMilieux_MISCELLANEOUSMedaka oryzias latipesphagophorevacuoleQHneurodegenerationAutophagosome cancer flux LC3 lysosome macroautophagy neurodegeneration phagophore stress vacuoleautophagy; autophagic processes; guidelines; autophagosome; cancer; flux; LC3; lysosome; macroautophagy; neurodegeneration; phagophore; stress; vacuolefluxmacroautophagystress.lysosomeAutophagosome; LC3; cancer; flux; lysosome; macroautophagy; neurodegeneration; phagophore; stress; vacuoleSettore BIO/17 - ISTOLOGIARC
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