Motor and Cerebellar Architectural Abnormalities during the Early Progression of Ataxia in a Mouse Model of SCA1 and How Early Prevention Leads to a Better Outcome Later in Life

Exposing developing cerebellar Purkinje neurons (PNs) to mutant Ataxin1 (ATXN1) in 82Q spinocerebellar ataxia type 1 (SCA1) mice disrupts motor behavior and cerebellar climbing fiber (CF) architecture from as early as 4 weeks of age. In contrast, if mutant ATXN1 expression is silenced until after ce...

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Detalles Bibliográficos
Autores principales: Ibrahim, Mohamed F., Power, Emmet M., Potapov, Kay, Empson, Ruth M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5611386/
https://www.ncbi.nlm.nih.gov/pubmed/28979190
http://dx.doi.org/10.3389/fncel.2017.00292

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5611386/
https://www.ncbi.nlm.nih.gov/pubmed/28979190
http://dx.doi.org/10.3389/fncel.2017.00292

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