Cell Cultures for the Study of Prion Diseases

Authors: K. Hobzová;  O. Janoušková
Authors‘ workplace: Ústav imunologie a mikrobiologie 1. LF UK v Praze
Published in: Cesk Slov Neurol N 2010; 73/106(4): 379-386
Category: Review Article


Transmissible spongiform encephalopathies, or prion diseases, comprise a group of fatal neurodegenerative disorders. Common to all these diseases is the accumulation of pathogenic prion protein isoforms in the brain. Much of the pathogenesis of prion diseases is studied in animal models. These experiments are time-consuming, expensive and ethically disturbing. In addition, animal models provide only limited scope for the study of biochemical aspects of these disorders in detail. Considerable efforts have been made to establish cell culture models supporting prion agent replication, but only a few permissive cell cultures have been found in which the stable propagation of prions can take place. Another restriction is associated with their ability to model only certain aspects of these diseases. The main advantage of cell culture is that it allows the rapid detection of prion infectivity and detailed study of pro­cesses linked to the propagation and spread of prions. Despite their limitations, cell cultures remain a powerful tool for the study of the mechanisms of prion propagation and the testing of anti-prion compounds that have much relevance to treatment of these diseases.

Key words:
transmission of spongiform encephalopathy – prions – cellular prion protein – cell cultures – experimental model


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