Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS

Inherited prion diseases are caused by autosomal dominant coding mutations in the human prion protein (PrP) gene (PRNP) and account for about 15% of human prion disease cases worldwide. The proposed mechanism is that the mutation predisposes to conformational change in the expressed protein, leading...

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Publicat a:PLoS Biol
Autors principals: Asante, Emmanuel A., Linehan, Jacqueline M., Tomlinson, Andrew, Jakubcova, Tatiana, Hamdan, Shyma, Grimshaw, Andrew, Smidak, Michelle, Jeelani, Asif, Nihat, Akin, Mead, Simon, Brandner, Sebastian, Wadsworth, Jonathan D. F., Collinge, John
Format: Artigo
Idioma:Inglês
Publicat: Public Library of Science 2020
Matèries:
Research Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC7282622/
https://ncbi.nlm.nih.gov/pubmed/32516343
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pbio.3000725
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