, Volume 36, Issue 4, pp 399-413
Date: 18 Apr 2014

Animal models of amyotrophic lateral sclerosis and Huntington’s disease

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Abstract

Amyotrophic lateral sclerosis (ALS) and Huntington’s disease (HD) are debilitating neurodegenerative conditions for which there is no effective cure. Genetic determinants of both diseases have been identified, providing insight into neuropathological mechanisms and opportunities for therapeutic intervention. Aggregation of mutant proteins is the most prominent phenotype of these neurodegenerative diseases as is the case in Alzheimer’s disease and Parkinson’s disease. Here we review transgenic animal models of ALS and HD in mouse, zebrafish, C. elegans, and Drosophila that have been developed to study different aspects of disease progression. We also cover some large mammal transgenic models that have been recently developed. To effectively tackle these conditions will likely require effective use of several of these animal models, as each offers distinct advantages and insights into disease pathology.