Transgenic mouse models for the study of prion diseases

Prions are unique agents that challenge the molecular biology dogma by transmitting information on the protein level. They cause neurodegenerative diseases that lack of any cure or treatment called transmissible spongiform encephalopathies. The function of the normal form of the prion protein, the exact mechanism of prion propagation between species as well as at the cellular level and neuron degeneration remains elusive. However, great amount of information known for all these aspects has been achieved thanks to the use of animal models and more precisely to transgenic mouse models. In this chapter, the main contributions of these powerful research tools in the prion field are revised.