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

Gallart Palau, Xavier Ramon; Ng, Chee-Hoe; Ribera i Calvet, Joan; Sze, Siu Kwan; Lim, Kah-Leong

doi:https://doi.org/10.1016/j.neulet.2016.05.006

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2016

Author

Gallart Palau, Xavier Ramon

Ng, Chee-Hoe

Ribera i Calvet, Joan

Sze, Siu Kwan

Lim, Kah-Leong

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.

URI

http://hdl.handle.net/10459.1/58918

DOI

https://doi.org/10.1016/j.neulet.2016.05.006

Is part of

Neuroscience Letters, 2016, num. 624, p. 47-52

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