Animal Models of Neurodegenerative Diseases

Abstract

The use of animals as models of neurodegenerative disorders has allowed the determination of biological targets and biomarkers of several diseases, has yielded new therapeutical perspectives, and is essential before performing novel clinical assays. This review discusses the nature, use, and limits of animal models and how to obtain them for several neurodegenerative disorders such as multiple system atrophy, amyotrophic lateral sclerosis, and Huntington's disease, with a special emphasis on Parkinson's and Alzheimer's diseases. When possible, rodent, invertebrate and primate models are presented and discussed in relation to human disease. Finally, we highlight discrepancies between animal models and human neuropathology leading to question the pertinence of some of these findings to human disorders probably because of the wide spectrum of parameters defining a disease. Another point raised by these studies is the growing necessity to standardize the experimental procedures used to obtain an animal model, housing and breeding conditions, assessments of phenotypes investigated and, ultimately the interpretation of results obtained and their relevance to the pathology.