Cell culture models of transmissible spongiform encephalopathies.
In this review, we describe the generation and use of cell culture models of transmissible spongiform encephalopathies, also known as prion diseases. These models include chronically prion-infected cell lines, as well as cultures expressing variable amounts of wild-type, mutated, or chimeric prion proteins. 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 occurring in transmissible spongiform encephalopathies and in the development of new therapeutic approaches of these diseases.
Béranger
F
F
Institut de Génétique Humaine, CNRS UPR 1142, 141, rue de la Cardonille, 34396 Montpellier Cedex 5, France.
Mangé
A
A
Solassol
J
J
Lehmann
S
S
D000818:Animals; D015153:Blotting, Western; D002478:Cells, Cultured; D004591:Electrophoresis, Polyacrylamide Gel; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D008954:Models, Biological; D009154:Mutation; D018621:PrPC Proteins / Q000378:metabolism; D018620:PrPSc Proteins / Q000378:metabolism; D017096:Prion Diseases / Q000378:metabolism* / Q000473:pathology* / Q000628:therapy*; D011328:Prions / Q000378:metabolism*; D011993:Recombinant Fusion Proteins / Q000378:metabolism
D018621:PrPC Proteins; D018620:PrPSc Proteins; D011328:Prions; D011993:Recombinant Fusion Proteins