Lack of a Clear Behavioral Phenotype in an Inducible FXTAS Mouse Model Despite the Presence of Neuronal FMRpolyG-Positive Aggregates

Lack of a Clear Behavioral Phenotype in an Inducible FXTAS Mouse Model Despite the Presence of Neuronal FMRpolyG-Positive Aggregates

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a rare neurodegenerative disorder caused by a 55–200 CGG repeat expansion in the 5′ untranslated region of the Fragile X Mental Retardation 1 (FMR1) gene. FXTAS is characterized by progressive cerebellar ataxia, Parkinsonism, intention tremors a...

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Bibliographic Details
Main Authors: Saif N. Haify, Ruchira S. D. Mankoe, Valerie Boumeester, Esmay C. van der Toorn, Rob F. M. Verhagen, Rob Willemsen, Renate K. Hukema, Laurens W. J. Bosman
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-12-01
Series:Frontiers in Molecular Biosciences
Subjects:
FXTAS
nuclear inclusions
mouse behavior
FMR1
repeat expansion
Online Access:https://www.frontiersin.org/articles/10.3389/fmolb.2020.599101/full

Internet

https://www.frontiersin.org/articles/10.3389/fmolb.2020.599101/full

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