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.

ions病毒是通过在蛋白质水平上传递信息来挑战分子生物学教条的独特药物。它们引起的神经退行性疾病缺乏任何治愈或治疗方法，称为传染性海绵状脑病。正常形式的normal病毒蛋白的功能，species病毒在物种之间以及在细胞水平和神经元变性中繁殖的确切机制仍然不清楚。但是，由于使用了动物模型，更确切地说是转基因小鼠模型，已经获得了所有这些方面的已知信息。在本章中，将对这些功能强大的研究工具在病毒领域的主要贡献进行修订。