Chapter 10:Animal Models for Huntington's Disease

Neurodegenerative diseases are very complex and their biological causes vary from one to another, but all of them have some characteristics in common: the progressive worsening of the symptoms and the degeneration of different components of the central nervous system that entails physical and mental disabilities. The generation of animal models to study these pathologies means a great advance in the knowledge of the metabolic pathways involved in the diseases, and helps to find out possible therapeutic targets. They are also the most physiological approach to perform global analysis of these diseases. Most of these animal models reproduce the pathogenesis by inserting or removing the mutation responsible for the disease from the animal genome. They can also be chemically generated when the cause of the disease is not genetic or known. The most used animals in laboratories are rodents but, lately, some laboratories are starting to use higher mammals to create new animal models to take advantage of the fact that their central nervous system is more similar to the human one, thus better resembling the human pathology.