Article
Plectin regulates the organization of glial fibrillary acidic protein in Alexander disease.
The American journal of pathology - 1 Mar 2006
Tian Rujin, Gregor Martin, Wiche Gerhard, Goldman James E
Abstract excerpt
Alexander disease (AxD) is a rare but fatal neurological disorder caused by mutations in the astrocyte-specific intermediate filament protein glial fibrillary acidic protein (GFAP). Histologically, AxD is characterized by cytoplasmic inclusion bodies called Rosenthal fibers (RFs), which contain GFAP, small heat shock proteins, and other undefined components. Here, we describe the expression of the cytoskeletal...
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