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Neuroscience. 2015 Mar 19;289:289-99. doi: 10.1016/j.neuroscience.2015.01.003. Epub 2015 Jan 14.

Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1.

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Department of Neuroscience, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, United States. Electronic address:
Department of Laboratory Medicine and Pathology, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, United States.
Neurology and Department of Cell and Molecular Biology, Northwestern University, 303 East Chicago Avenue, Chicago, IL 60611, United States.


Spinocerebellar ataxia type 1 (SCA1) is an incurable, dominantly inherited neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion in the protein ataxin-1 (ATXN1). While analysis of human autopsy material indicates significant glial pathology in SCA1, previous research has focused on characterizing neuronal dysfunction. In this study, we characterized astrocytic and microglial response in SCA1 using a comprehensive array of mouse models. We have discovered that astrocytes and microglia are activated very early in SCA1 pathogenesis even when mutant ATXN1 expression was limited to Purkinje neurons. Glial activation occurred in the absence of neuronal death, suggesting that glial activation results from signals emanating from dysfunctional neurons. Finally, in all different models examined glial activation closely correlated with disease progression, supporting the development of glial-based biomarkers to follow disease progression.


SCA1; astrocytes; glia; microglia; neurodegeneration; spinocerebellar ataxia type 1

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