Animal models of amyotrophic lateral sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the selective death of motor neurons. Although ALS is predominantly a disease of motor system degeneration, cognitive impairments have also been reported. In order to determine the pathogenic mechanisms leading to this neurodegeneration, a large number of different animal models have helped to get insights. These animal models contain mutated genes (spontaneous or induced) or (over)express (mutant) genes, and recapitulate important aspect of this neurodegenerative disease. Although the exact mechanisms are not yet elucidated, animal models have been of great value to delineate potential pathogenic mechanisms that are involved in, and/or are important in causing selective motor neuron degeneration. Moreover, these animal models can be used as first screening assays to select potential therapeutics to treat this fatal disease. © 2011 Springer Science+Business Media, LLC.