Article
Phenotypic conversions of "protoplasmic" to "reactive" astrocytes in Alexander disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 24 Apr 2013
Sosunov Alexander A, Guilfoyle Eileen, Wu Xiaoping, McKhann Guy M, Goldman James E
Abstract excerpt
Alexander Disease (AxD) is a primary disorder of astrocytes, caused by heterozygous mutations in GFAP, which encodes the major astrocyte intermediate filament protein, glial fibrillary acidic protein (GFAP). Astrocytes in AxD display hypertrophy, massive increases in GFAP, and the accumulation of Rosenthal fibers, cytoplasmic protein inclusions containing GFAP, and small heat shock proteins. To study the effects...
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