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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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
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Online Access:https://hdl.handle.net/10356/84611
http://hdl.handle.net/10220/41891
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Institution: Nanyang Technological University
Language: English
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spelling sg-ntu-dr.10356-846112023-02-28T17:01:10Z Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis Gallart-Palau, Xavier Ng, Chee-Hoe Ribera, Joan Sze, Siu Kwan Lim, Kah-Leong School of Biological Sciences FALS SOD1G93A 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. Accepted version 2016-12-19T08:40:08Z 2019-12-06T15:48:19Z 2016-12-19T08:40:08Z 2019-12-06T15:48:19Z 2016 Journal Article 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. 0304-3940 https://hdl.handle.net/10356/84611 http://hdl.handle.net/10220/41891 10.1016/j.neulet.2016.05.006 en Neuroscience Letters © 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]. 19 p. application/pdf
institution Nanyang Technological University
building NTU Library
continent Asia
country Singapore
Singapore
content_provider NTU Library
collection DR-NTU
language English
topic FALS
SOD1G93A
spellingShingle FALS
SOD1G93A
Gallart-Palau, Xavier
Ng, Chee-Hoe
Ribera, Joan
Sze, Siu Kwan
Lim, Kah-Leong
Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
description 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.
author2 School of Biological Sciences
author_facet School of Biological Sciences
Gallart-Palau, Xavier
Ng, Chee-Hoe
Ribera, Joan
Sze, Siu Kwan
Lim, Kah-Leong
format Article
author Gallart-Palau, Xavier
Ng, Chee-Hoe
Ribera, Joan
Sze, Siu Kwan
Lim, Kah-Leong
author_sort Gallart-Palau, Xavier
title Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
title_short Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
title_full Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
title_fullStr Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
title_full_unstemmed Drosophila expressing human SOD1 successfully recapitulates mitochondrial phenotypic features of familial Amyotrophic Lateral Sclerosis
title_sort drosophila expressing human sod1 successfully recapitulates mitochondrial phenotypic features of familial amyotrophic lateral sclerosis
publishDate 2016
url https://hdl.handle.net/10356/84611
http://hdl.handle.net/10220/41891
_version_ 1759853783788027904