TY  - JOUR
AU  - Matamoros-Angles, Andreu
AU  - Gayosso, Lucía Mayela
AU  - Richaud-Patin, Yvonne
AU  - di Domenico, Angelique
AU  - Vergara, Cristina
AU  - Hervera, Arnau
AU  - Sousa, Amaya
AU  - Fernández-Borges, Natalia
AU  - Consiglio, Antonella
AU  - Gavín, Rosalina
AU  - López de Maturana, Rakel
AU  - Ferrer, Isidro
AU  - López de Munain, Adolfo
AU  - Raya, Ángel
AU  - Castilla, Joaquín
AU  - Sánchez-Pernaute, Rosario
AU  - Del Río, José Antonio
PY  - 2017
DO  - 10.1007/s12035-017-0506-6
UR  - http://hdl.handle.net/10668/11161
T2  - Molecular neurobiology
AB  - 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...
LA  - en
KW  - Cellular prion protein
KW  - Gerstmann-Sträussler-Scheinker
KW  - Induced pluripotent stem cells
KW  - Tau
KW  - Astrocytes
KW  - Base Sequence
KW  - Brain
KW  - Cell Differentiation
KW  - Cells, Cultured
KW  - Female
KW  - Gerstmann-Straussler-Scheinker Disease
KW  - Gliosis
KW  - Humans
KW  - Induced Pluripotent Stem Cells
KW  - Middle Aged
KW  - Mitochondria
KW  - Mutation
KW  - Neurons
KW  - Phosphorylation
KW  - Prion Proteins
KW  - tau Proteins
TI  - iPS Cell Cultures from a Gerstmann-Sträussler-Scheinker Patient with the Y218N PRNP Mutation Recapitulate tau Pathology.
TY  - research article
VL  - 55
ER  -