Cell culture models of transmissible spongiform encephalopathies

In this review, we describe the generation and use of cell culture models o f transmissible spongiform encephalopathies, also known as prion diseases. These models include chronically prion-infected cell lines, as well as cult ures expressing variable amounts of wild-type, mutated, or chimeric prion p roteins. These cell lines have been widely used to investigate the biology of both the normal and the pathological isoform of the prion protein. They have also contributed to the, comprehension of the pathogenic processes occ urring in transmissible spongiform encephalopathies and in the development of new therapeutic approaches of these diseases. (C) 2001 Elsevier Science.