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Profoundly different prion diseases in knock-in mice carrying single PrP codon substitutions associated with human diseases [Neuroscience]

In man, mutations in different regions of the prion protein (PrP) are associated with infectious neurodegenerative diseases that have remarkably different clinical signs and neuropathological lesions. To explore the roots of this phenomenon, we created a knock-in mouse model carrying the mutation associated with one of these diseases [Creutzfeldt–Jakob disease...

Authors:   Walker S. Jackson; Andrew W. Borkowski; Nicki E. Watson; Oliver D. King; Henryk Faas; Alan Jasanoff; Susan Lindquist
Journal:   Proceedings of the National Academy of Sciences current issue
Volume:   110
edition:   36
Year:   2013
Pages:   14759
DOI:   10.1073/pnas.1312006110
Publication date:   03-Sep-2013
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