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Article

Altered synaptic and firing properties of cerebellar Purkinje cells in a mouse model of ARSACS.

The Journal of physiology - 1 Sept 2018

Ady Visou, Toscano-Márquez Brenda, Nath Moushumi, Chang Philip K, Hui Jeanette, Cook Anna, Charron François, Larivière Roxanne, Brais Bernard, McKinney R Anne, Watt Alanna J

Abstract excerpt

KEY POINTS: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is an early-onset neurodegenerative human disease characterized in part by ataxia and Purkinje cell loss in anterior cerebellar lobules. A knock-out mouse model has been developed that recapitulates several features of ARSACS. Using this ARSACS mouse model, we report changes in synaptic input and intrinsic firing in cerebellar Purkinje...

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PMID: 29928778
DOI: 10.1113/JP275902
PMCID: PMC6117548

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