Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis

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2016
Author
Gallart Palau, Xavier Ramon
Ng, Chee-Hoe
Ribera i Calvet, Joan
Sze, Siu Kwan
Lim, Kah-Leong
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Gallart Palau, Xavier Ramon; Ng, Chee-Hoe; Ribera i Calvet, Joan; Sze, Siu Kwan; Lim, Kah-Leong; . (2016) . Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis. Neuroscience Letters, 2016, num. 624, p. 47-52. https://doi.org/10.1016/j.neulet.2016.05.006.
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Abstract
Mitochondrial 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.
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http://hdl.handle.net/10459.1/58918
DOI
https://doi.org/10.1016/j.neulet.2016.05.006
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Neuroscience Letters, 2016, num. 624, p. 47-52
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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