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...
Main Authors: | , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2015-10-01
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Series: | Neurobiology of Disease |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996115002223 |