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dc.contributor.authorGallart Palau, Xavier Ramon
dc.contributor.authorNg, Chee-Hoe
dc.contributor.authorRibera i Calvet, Joan
dc.contributor.authorSze, Siu Kwan
dc.contributor.authorLim, Kah-Leong
dc.date.accessioned2017-01-09T18:27:47Z
dc.date.available2017-06-30T22:38:41Z
dc.date.issued2016
dc.identifier.issn0304-3940
dc.identifier.urihttp://hdl.handle.net/10459.1/58918
dc.description.abstractMitochondrial pathology is a seminal pathogenic hallmark of familial amyotrophic lateral sclerosis (FALS) which is extensively manifested by human patients and mutant SOD1G93A mammalian models. Rodents expressing human FALS-associated mutations successfully mimic several human disease features; although they are not as amenable to genetic and therapeutic compound screenings as non-mammalian models. In this study, we report a newly generated and characterized Drosophila model that expresses human SOD1G93A in muscle fibers. Presence of SOD1G93A in thoracic muscles causes mitochondrial pathology and impairs normal motor behavior in these flies. Use of this new FALS-24B-SOD1G93A fly model holds promise for better understanding of the mitochondrial affectation process in FALS and for the discovery of novel therapeutic compounds able to reverse mitochondrial dysfunction in this fatal disease.
dc.format.mimetypeapplication/pdf
dc.language.isoeng
dc.publisherElsevier
dc.relation.isformatofVersió postprint del document publicat a: https://doi.org/10.1016/j.neulet.2016.05.006
dc.relation.ispartofNeuroscience Letters, 2016, num. 624, p. 47-52
dc.rightscc-by-nc-nd (c) Elsevier, 2016
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subjectALS
dc.subjectSuperoxide dismutase 1 (SOD 1)
dc.subjectMitochondria
dc.subject.classificationEsclerosi lateral amiotròfica
dc.subject.classificationDrosòfila
dc.subject.classificationMitocondris
dc.subject.classificationSuperòxid dismutasa
dc.subject.otherAmyotrophic lateral sclerosis
dc.subject.otherDrosophila
dc.subject.otherMitochondria
dc.subject.otherSuperoxide dismutase
dc.titleDrosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis
dc.typeinfo:eu-repo/semantics/article
dc.date.updated2017-01-09T18:27:48Z
dc.identifier.idgrec025152
dc.type.versioninfo:eu-repo/semantics/acceptedVersion
dc.identifier.doihttps://doi.org/10.1016/j.neulet.2016.05.006
dc.rights.accessRightsinfo:eu-repo/semantics/openAccess


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cc-by-nc-nd (c) Elsevier, 2016
Except where otherwise noted, this item's license is described as cc-by-nc-nd (c) Elsevier, 2016