Journal
CEREBELLUM
Volume 14, Issue 1, Pages 8-11Publisher
SPRINGER
DOI: 10.1007/s12311-014-0605-0
Keywords
Spinocerebellar ataxia type 1; SCA1; Neurodegeneration; Glia; Astrocytes; Glutamate transporter; Excitotoxicity
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Funding
- University of Minnesota Department of Neuroscience
- Institute for the Translational Neuroscience
- Minnesota Medical Foundation
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Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion in the protein ATXN1. We have previously demonstrated that astrocytic activation occurs early in pathogenesis, correlates with disease progression, and can occur when mutant ATXN1 expression is limited to Purkinje neurons. We now show that expression of glutamate and aspartate transporter, GLAST, is decreased in cerebellar astrocytes in a mouse model of SCA1. This decrease occurs in non-cell autonomous manner late in disease and correlates well with the loss of Purkinje neurons. Astrogliosis or decreased neuronal activity does not correlate with diminished GLAST expression. In addition, Bergmann glia remain capable of transcriptional upregulation of GLAST in response to improvement in Purkinje neurons supporting the notion of active neuron-glia crosstalk in disease.
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