Journal
ACTA NEUROPATHOLOGICA COMMUNICATIONS
Volume 4, Issue -, Pages -Publisher
BMC
DOI: 10.1186/s40478-016-0337-0
Keywords
Alexander disease (AxD); Glial fibrillary acidic protein (GFAP); Induced pluripotent stem cells (iPSCs); Disease modeling; Astrocytes; Rosenthal fibers; Heat-shock protein; Alpha-crystallin; Cytokine; Inflammatory response; Inherited astrocytopathy
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Funding
- Core Center for iPS Cell Research of Research Center Network for Realization of Regenerative Medicine from AMED
- Mochida Memorial Foundation for Medical and Pharmaceutical Research
- Daiichi Sankyo Foundation of Life Science
- Neurological and Psychiatry Disorders of NCNP [24-9]
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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.
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