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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...

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Principais autores: Gry Hoem, Kenneth Bowitz Larsen, Aud Øvervatn, Andreas Brech, Trond Lamark, Eva Sjøttem, Terje Johansen
פורמט: Artigo
שפה:Inglês
יצא לאור: Frontiers Media S.A. 2019-03-01
סדרה:Frontiers in Genetics
נושאים:
FXTAS
CGG repeat expansion
RNA hairpin
RAN translation
polyglycine
protein degradation
גישה מקוונת:https://www.frontiersin.org/article/10.3389/fgene.2019.00249/full
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