Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis

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; alth...

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Bibliographic Details
Main Authors: Gallart-Palau, Xavier, Ng, Chee-Hoe, Ribera, Joan, Sze, Siu Kwan, Lim, Kah-Leong
Other Authors: School of Biological Sciences
Format: Article
Language:English
Published: 2016
Subjects:
Online Access:https://hdl.handle.net/10356/84611
http://hdl.handle.net/10220/41891
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Institution: Nanyang Technological University
Language: English
Description
Summary: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.