Cargando…
A naturally occurring variant of the human prion protein completely prevents prion disease
Mammalian prions, transmissible agents causing lethal neurodegenerative diseases, are composed of assemblies of misfolded cellular prion protein (PrP) (1). A novel PrP variant, G127V, was under positive evolutionary selection during the epidemic of kuru, an acquired prion disease epidemic of the For...
Autores principales: | Asante, Emmanuel A., Smidak, Michelle, Grimshaw, Andrew, Houghton, Richard, Tomlinson, Andrew, Jeelani, Asif, Jakubcova, Tatiana, Hamdan, Shyma, Richard-Londt, Angela, Linehan, Jacqueline M., Brandner, Sebastian, Alpers, Michael, Whitfield, Jerome, Mead, Simon, Wadsworth, Jonathan D.F., Collinge, John |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
2015
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4486072/ https://www.ncbi.nlm.nih.gov/pubmed/26061765 http://dx.doi.org/10.1038/nature14510 |
Ejemplares similares
-
Inherited Prion Disease A117V Is Not Simply a Proteinopathy but Produces Prions Transmissible to Transgenic Mice Expressing Homologous Prion Protein
por: Asante, Emmanuel A., et al.
Publicado: (2013) -
Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS
por: Asante, Emmanuel A., et al.
Publicado: (2020) -
Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS
por: Asante, Emmanuel A., et al.
Publicado: (2015) -
Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins
por: Asante, Emmanuel A., et al.
Publicado: (2009) -
The origin of the prion agent of kuru: molecular and biological strain typing
por: Wadsworth, Jonathan D.F., et al.
Publicado: (2008)