Please use this identifier to cite or link to this item: https://hdl.handle.net/10356/84611
Title: Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
Authors: Gallart-Palau, Xavier
Ng, Chee-Hoe
Ribera, Joan
Sze, Siu Kwan
Lim, Kah-Leong
Keywords: FALS
SOD1G93A
Issue Date: 2016
Source: Gallart-Palau, X., Ng, C. -H., Ribera, J., Sze, S. K., & Lim, K. -L. (2016). Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis. Neuroscience Letters, 624, 47-52.
Series/Report no.: Neuroscience Letters
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: https://hdl.handle.net/10356/84611
http://hdl.handle.net/10220/41891
ISSN: 0304-3940
DOI: 10.1016/j.neulet.2016.05.006
Rights: © 2016 Elsevier Ireland Ltd. This is the author created version of a work that has been peer reviewed and accepted for publication by Neuroscience Letters, Elsevier. It incorporates referee’s comments but changes resulting from the publishing process, such as copyediting, structural formatting, may not be reflected in this document. The published version is available at: [http://dx.doi.org/10.1016/j.neulet.2016.05.006].
Fulltext Permission: open
Fulltext Availability: With Fulltext
Appears in Collections:SBS Journal Articles

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