Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR. Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport. Nat Neurosci. 2006 Oct;9(10):1302-11. PubMed.
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This result is important because it provides a striking example that neurodegeneration is not always the result of a primary problem in the neuron, but that the neuron can die secondary to a primary problem with the glial cells. Almost always people tend to think of the glial cell changes as secondary to the neuron loss. In fact, there are already other examples of this in retinal degenerations. In some forms of photoreceptor degeneration, for instance, the primary defect is in the retinal pigment epithelium cells, which leads ultimately to death of photoreceptors (Feng et al., 2002).
References:
Feng W, Yasumura D, Matthes MT, LaVail MM, Vollrath D. Mertk triggers uptake of photoreceptor outer segments during phagocytosis by cultured retinal pigment epithelial cells. J Biol Chem. 2002 May 10;277(19):17016-22. PubMed.
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