Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.

Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.

Prions are infectious agents causing fatal neurodegenerative diseases of humans and animals. In humans, these have sporadic, acquired and inherited aetiologies. The inherited prion diseases are caused by one of over 30 coding mutations in the human prion protein (PrP) gene (PRNP) and many of these g...

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Bibliographic Details
Main Authors: Emmanuel A Asante, Jacqueline M Linehan, Michelle Smidak, Andrew Tomlinson, Andrew Grimshaw, Asif Jeelani, Tatiana Jakubcova, Shyma Hamdan, Caroline Powell, Sebastian Brandner, Jonathan D F Wadsworth, John Collinge
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2013-01-01
Series:PLoS Pathogens
Online Access:http://europepmc.org/articles/PMC3784465?pdf=render

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http://europepmc.org/articles/PMC3784465?pdf=render

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