Title |
Modeling Alexander disease with patient iPSCs reveals cellular and molecular pathology of astrocytes
|
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Published in |
Acta Neuropathologica Communications, July 2016
|
DOI | 10.1186/s40478-016-0337-0 |
Pubmed ID | |
Authors |
Takayuki Kondo, Misato Funayama, Michiyo Miyake, Kayoko Tsukita, Takumi Era, Hitoshi Osaka, Takashi Ayaki, Ryosuke Takahashi, Haruhisa Inoue |
Abstract |
Alexander disease is a fatal neurological illness characterized by white-matter degeneration and formation of Rosenthal fibers, which contain glial fibrillary acidic protein as astrocytic inclusion. Alexander disease is mainly caused by a gene mutation encoding glial fibrillary acidic protein, although the underlying pathomechanism remains unclear. We established induced pluripotent stem cells from Alexander disease patients, and differentiated induced pluripotent stem cells into astrocytes. Alexander disease patient astrocytes exhibited Rosenthal fiber-like structures, a key Alexander disease pathology, and increased inflammatory cytokine release compared to healthy control. These results suggested that Alexander disease astrocytes contribute to leukodystrophy and a variety of symptoms as an inflammatory source in the Alexander disease patient brain. Astrocytes, differentiated from induced pluripotent stem cells of Alexander disease, could be a cellular model for future translational medicine. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Japan | 1 | 33% |
United States | 1 | 33% |
Unknown | 1 | 33% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 3 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 67 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Researcher | 17 | 25% |
Student > Ph. D. Student | 9 | 13% |
Student > Master | 6 | 9% |
Other | 5 | 7% |
Student > Doctoral Student | 4 | 6% |
Other | 11 | 16% |
Unknown | 15 | 22% |
Readers by discipline | Count | As % |
---|---|---|
Neuroscience | 17 | 25% |
Agricultural and Biological Sciences | 12 | 18% |
Medicine and Dentistry | 10 | 15% |
Biochemistry, Genetics and Molecular Biology | 6 | 9% |
Unspecified | 2 | 3% |
Other | 4 | 6% |
Unknown | 16 | 24% |