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

NEUROSCIENCE LETTERS

卷:624

Article

Gallart-Palau, Xavier^1,2,3  Ng, Chee-Hoe^5,6  Ribera, Joan^1,2  Sze, Siu Kwan³  Lim, Kah-Leong^4,5,6 

[1] Univ Lleida, Sch Med, Dept Expt Med, Lleida 25198, Catalonia, Spain

[2] Inst Recerca Biomed Lleida IRBLLEIDA, Lleida 25198, Catalonia, Spain

[3] Nanyang Technol Univ, Sch Biol Sci, Singapore 637551, Singapore

[4] Natl Univ Singapore, Dept Physiol, Singapore 117543, Singapore

[5] Natl Neurosci Inst, Dept Res, Singapore 308433, Singapore

[6] Duke NUS Grad Med Sch, Singapore 169857, Singapore

关键词: FALS;    SOD1(G93A);    Mitochondria;    Drosophila;   

DOI  :  10.1016/j.neulet.2016.05.006

来源: Elsevier

【 摘 要 】

Mitochondrial pathology is a seminal pathogenic hallmark of familial amyotrophic lateral sclerosis (FALS) which is extensively manifested by human patients and mutant SOD1(G93A) 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 SOD1(G93A) in muscle fibers. Presence of SOD1(G93A) in thoracic muscles causes mitochondrial pathology and impairs normal motor behavior in these flies. Use of this new FALS-24B-SOD1(G93A) 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. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

【 授权许可】

Free