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Significance The mechanisms underlying the degeneration of specific cellular populations in various neurodegenerative disorders remain unknown. Here, we show that the polyQ expansion of ataxin-1 activates the Wnt-β-catenin signaling pathway… Click to show full abstract
Significance The mechanisms underlying the degeneration of specific cellular populations in various neurodegenerative disorders remain unknown. Here, we show that the polyQ expansion of ataxin-1 activates the Wnt-β-catenin signaling pathway in various cell types, including Purkinje cells and Bergmann glia, in the cerebellum of spinocerebellar ataxia type 1 (SCA1) mouse models. We used conditional mouse genetics to activate and silence this pathway in different cell types and found elevated activity of this signaling pathway impacted Bergmann glia and Purkinje cell populations differently. This study highlights the important role of the Wnt-β-catenin signaling pathway in glial cell types for SCA1 pathogenesis.
Journal Title: Proceedings of the National Academy of Sciences of the United States of America
Year Published: 2022
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