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• W2062327509 abstract "Alexander disease (AxD) is a neurodegenerative disorder with prominent white matter degeneration and the presence of Rosenthal fibers containing aggregates of glial fibrillary acidic protein ( GFAP ), and small stress proteins HSP 27 and αB‐crystallin, and widespread reactive gliosis. AxD is caused by mutations in GFAP , the main astrocyte intermediate filament protein. We previously showed that intermediate filament protein synemin is upregulated in reactive astrocytes after neurotrauma. Here, we examined immunohistochemically the presence of synemin in reactive astrocytes and Rosenthal fibers in two patients with AxD. There was an abundance of GFAP ‐positive Rosenthal fibers and widespread reactive gliosis in the white matter and subpial regions. Many of the GFAP ‐positive reactive astrocytes were positive for synemin, and synemin was also present in Rosenthal fibers. We show that synemin is expressed in reactive astrocytes in AxD, and is also present in Rosenthal fibers. The potential role of synemin in AxD pathogenesis remains to be investigated." @default.
• W2062327509 created "2016-06-24" @default.
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• W2062327509 date "2013-04-18" @default.
• W2062327509 modified "2023-10-16" @default.
• W2062327509 title "Synemin is expressed in reactive astrocytes and Rosenthal fibers in Alexander disease" @default.
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• W2062327509 doi "https://doi.org/10.1111/apm.12088" @default.
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