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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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Detalhes bibliográficos
Main Authors: Asante, Emmanuel A., Linehan, Jacqueline M., Smidak, Michelle, Tomlinson, Andrew, Grimshaw, Andrew, Jeelani, Asif, Jakubcova, Tatiana, Hamdan, Shyma, Powell, Caroline, Brandner, Sebastian, Wadsworth, Jonathan D. F., Collinge, John
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2013
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3784465/
https://ncbi.nlm.nih.gov/pubmed/24086135
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.ppat.1003643
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