Astroglial-targeted expression of the fragile X CGG repeat premutation in mice yields RAN translation, motor deficits and possible evidence for cell-to-cell propagation of FXTAS pathology

The fragile X premutation is a CGG trinucleotide repeat expansion between 55 and 200 repeats in the 5′-untranslated region of the fragile X mental retardation 1 (FMR1) gene. Human carriers of the premutation allele are at risk of developing the late-onset neurodegenerative disorder, fragile X-associ...

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Detalles Bibliográficos
Autores principales: Wenzel, H. Jürgen, Murray, Karl D., Haify, Saif N., Hunsaker, Michael R., Schwartzer, Jared J., Kim, Kyoungmi, La Spada, Albert R., Sopher, Bryce L., Hagerman, Paul J., Raske, Christopher, Severijnen, Lies-Anne W.F.M., Willemsen, Rob, Hukema, Renate K., Berman, Robert F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390634/
https://www.ncbi.nlm.nih.gov/pubmed/30808398
http://dx.doi.org/10.1186/s40478-019-0677-7

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