iPS Cell Cultures from a Gerstmann-Sträussler-Scheinker Patient with the Y218N PRNP Mutation Recapitulate tau Pathology

Gerstmann-Sträussler-Scheinker (GSS) syndrome is a fatal autosomal dominant neurodegenerative prionopathy clinically characterized by ataxia, spastic paraparesis, extrapyramidal signs and dementia. In some GSS familiar cases carrying point mutations in the PRNP gene, patients also showed comorbid ta...

Descripción completa

Detalles Bibliográficos
Autores principales: Matamoros-Angles, Andreu, Gayosso, Lucía Mayela, Richaud-Patin, Yvonne, di Domenico, Angelique, Vergara, Cristina, Hervera, Arnau, Sousa, Amaya, Fernández-Borges, Natalia, Consiglio, Antonella, Gavín, Rosalina, López de Maturana, Rakel, Ferrer, Isidro, López de Munain, Adolfo, Raya, Ángel, Castilla, Joaquín, Sánchez-Pernaute, Rosario, del Río, José Antonio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5842509/
https://www.ncbi.nlm.nih.gov/pubmed/28466265
http://dx.doi.org/10.1007/s12035-017-0506-6