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...

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Publicado en:Front Genet
Autores principales: Hoem, Gry, Bowitz Larsen, Kenneth, Øvervatn, Aud, Brech, Andreas, Lamark, Trond, Sjøttem, Eva, Johansen, Terje
Formato: Artigo
Lenguaje:Inglês
Publicado: Frontiers Media S.A. 2019
Materias:
Genetics
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6447689/
https://ncbi.nlm.nih.gov/pubmed/30984240
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fgene.2019.00249
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