title: A fruitful endeavor: modeling ALS in the fruit fly.
abstract: 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 learned using Drosophila models pertaining to ALS research. This article is part of a Special Issue entitled ALS complex pathogenesis.
authors.affiliation: Department of Pediatrics, Child Neurology and Neurobiology, Children׳s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA 15224, USA; Human Genetics Graduate Program, University of Pittsburgh School of Public Health, Pittsburgh, PA 15261, USA.
authors.forename: Ian
authors.initials: I
authors.lastname: Casci
authors.affiliation: Department of Pediatrics, Child Neurology and Neurobiology, Children׳s Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA 15224, USA. Electronic address: udai.pandey@chp.edu.
authors.forename: Udai Bhan
authors.initials: UB
authors.lastname: Pandey
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000503:physiopathology*; D000818:Animals; D030801:Animals, Genetically Modified; D004195:Disease Models, Animal; D004331:Drosophila melanogaster / Q000235:genetics / Q000502:physiology*; D006801:Humans
keywords: ALS; Amyotrophic lateral sclerosis; Animal model; C9ORF72; Drosophila; FTLD; FUS; Fly model; Motor neuron disease; Neurodegeneration; SOD1; TDP-43; VCP