The FMRpolyGlycine Protein Mediates Aggregate Formation and Toxicity Independent of the CGG mRNA Hairpin in a Cellular Model for FXTAS

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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Bibliographic Details
Main Authors: Gry Hoem, Kenneth Bowitz Larsen, Aud Øvervatn, Andreas Brech, Trond Lamark, Eva Sjøttem, Terje Johansen
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-03-01
Series:Frontiers in Genetics
Subjects:
FXTAS
CGG repeat expansion
RNA hairpin
RAN translation
polyglycine
protein degradation
Online Access:https://www.frontiersin.org/article/10.3389/fgene.2019.00249/full

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https://www.frontiersin.org/article/10.3389/fgene.2019.00249/full

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