Article
Spinocerebellar ataxia type 7 cerebellar disease requires the coordinated action of mutant ataxin-7 in neurons and glia, and displays non-cell-autonomous bergmann glia degeneration.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 9 Nov 2011
Furrer Stephanie A, Mohanachandran Mathini S, Waldherr Sarah M, Chang Christopher, Damian Vincent A, Sopher Bryce L, Garden Gwenn A, La Spada Albert R
Abstract excerpt
Spinocerebellar ataxia type 7 (SCA7) is a dominantly inherited disorder characterized by cerebellum and brainstem neurodegeneration. SCA7 is caused by a CAG/polyglutamine (polyQ) repeat expansion in the ataxin-7 gene. We previously reported that directed expression of polyQ-ataxin-7 in Bergmann glia (BG) in transgenic mice leads to ataxia and non-cell-autonomous Purkinje cell (PC) degeneration. To further define...
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