期刊
APMIS
卷 122, 期 1, 页码 76-80出版社
WILEY-BLACKWELL
DOI: 10.1111/apm.12088
关键词
Astrocytes; GFAP; intermediate filaments; Alexander disease; neurodegenerative diseases
资金
- Swedish Medical Research Council [11548]
- AFA Insurance
- Swedish Stroke Foundation
- Torsten Foundation
- Ragnar Soderberg Foundation
- Swedish Society for Medicine
- Region of Vastra Gotaland
- Frimurare Foundation
- Amlov's Foundation
- Sten A. Olsson Foundation for Research and Culture
- Foundation Edit Jacobson's Donation Fund
- Trygg-Hansa
- Hjarnfonden
- ALF Goteborg [11392]
- EU [237956]
- TargetBraIn [279017]
- NanoNet COST Action [BM1002]
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 HSP27 and B-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.
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