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The FMRpolyGlycine Protein Mediates Aggregate Formation and Toxicity Independent of the CGG mRNA Hairpin in a Cellular Model for FXTAS
Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by a CGG-repeat expansion in the 5′ UTR of the FMR1 gene on the X-chromosome. Both elevated levels of the expanded FMR1 mRNA and aberrant expression of a polyglycine protein (FMRpolyG) from the CGG-repeat regi...
Autores principales: | Hoem, Gry, Bowitz Larsen, Kenneth, Øvervatn, Aud, Brech, Andreas, Lamark, Trond, Sjøttem, Eva, Johansen, Terje |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6447689/ https://www.ncbi.nlm.nih.gov/pubmed/30984240 http://dx.doi.org/10.3389/fgene.2019.00249 |
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