Journal
BRAIN RESEARCH
Volume 1607, Issue -, Pages 47-74Publisher
ELSEVIER
DOI: 10.1016/j.brainres.2014.09.064
Keywords
Amyotrophic lateral sclerosis; ALS; Drosophila; Fly model; Animal model; Neurodegeneration; Motor neuron disease; FTLD; SOD1; TDP-43; FUS; C9ORF72; VCP
Categories
Funding
- Robert Packard Center
- Amyotrophic Lateral Sclerosis Association
- National Institute of Health [R01-NS081303]
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For over a century Drosophila melanogaster, commonly known as the fruit fly, has been instrumental in genetics research and disease modeling. In more recent years, it has been a powerful tool for modeling and studying neurodegenerative diseases, including the devastating and fatal amyotrophic lateral sclerosis (ALS). The success of this model organism in ALS research comes from the availability of tools to manipulate gene/protein expression in a number of desired cell-types, and the subsequent recapitulation of cellular and molecular phenotypic features of the disease. Several Drosophila models have now been developed for studying the roles of ALS-associated genes in disease pathogenesis that allowed us to understand the molecular pathways that lead to motor neuron degeneration in ALS patients. Our primary goal in this review is to highlight the lessons we have leamed using Drosophila models pertaining to ALS research. This article is part of a Special Issue entitled ALS complex pathogenesis. (C) 2014 Elsevier B.V. All rights reserved.
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