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Published in:
2013 | OriginalPaper | Chapter
Synthetic Infectious Prions
Authors : G. Legname, N. Le Tran Thanh
Published in: 4th International Conference on Biomedical Engineering in Vietnam
Publisher: Springer Berlin Heidelberg
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Prion diseases are fatal and incurable neurodegenerative maladies of unique etiology. These disorders have attracted considerable attention for their unique infectivity pathogenic mechanism. Here we describe the milestone studies that have lead to the demonstration that prions are solely constituted of proteins. The formal demonstration has been achieved through several papers describing unconvertible evidence that recombinant prion protein refolded into amyloid fibrils can contain infectious species that lead to disease in animal models of prion coversion and replication. These molecules have been named as synthetic infectious prions.