Mayhem of the multiple mechanisms: Modelling neurodegeneration in prion disease

Abstract

This review examines recent attempts to advance the understanding of the mechanism by which neurones die in prion disease. Prion diseases or transmissible spongiform encephalopathies are characterized by the conversion of a normal glycoprotein, the prion protein, to a protease-resistant form that is suggested to be both the infectious agent and the cause of the rapid neurodegeneration in the disease. Death of the patient results from this widespread neuronal loss. Thus understanding the mechanism by which the abnormal form of the prion protein causes neuronal death might lead to treatments that would prevent the life-threatening nature of these diseases.