Aberrant Purkinje cell activity is the cause of dystonia in a shRNA-based mouse model of Rapid Onset Dystonia–Parkinsonism

Loss-of-function mutations in the α3 isoform of the sodium pump are responsible for Rapid Onset Dystonia–Parkinsonism (RDP). A pharmacologic model of RDP replicates the most salient features of RDP, and implicates both the cerebellum and basal ganglia in the disorder; dystonia is associated with abe...

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Bibliographic Details
Main Authors: Rachel Fremont, Ambika Tewari, Kamran Khodakhah
Format: Article
Language:English
Published: Elsevier 2015-10-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996115002223