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

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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Bibliographic Details
Main Authors: Mohamed F. Ibrahim, Emmet M. Power, Kay Potapov, Ruth M. Empson
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-09-01
Series:Frontiers in Cellular Neuroscience
Subjects:
cerebellum
ataxias
climbing fibers
Purkinje cells
mouse models
Online Access:http://journal.frontiersin.org/article/10.3389/fncel.2017.00292/full

Internet

http://journal.frontiersin.org/article/10.3389/fncel.2017.00292/full

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