Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS

Expansion of a (G(4)C(2))(n) repeat in C9orf72 causes amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the link of the five repeat-encoded dipeptide repeat (DPR) proteins to neuroinflammation, TDP-43 pathology, and neurodegeneration is unclear. Poly-PR is most toxic in vitr...

詳細記述

保存先:
書誌詳細
出版年:Acta Neuropathol
主要な著者: LaClair, Katherine D., Zhou, Qihui, Michaelsen, Meike, Wefers, Benedikt, Brill, Monika S., Janjic, Aleksandar, Rathkolb, Birgit, Farny, Daniel, Cygan, Mikolaj, de Angelis, Martin Hrabe, Wurst, Wolfgang, Neumann, Manuela, Enard, Wolfgang, Misgeld, Thomas, Arzberger, Thomas, Edbauer, Dieter
フォーマット: Artigo
言語:Inglês
出版事項: Springer Berlin Heidelberg 2020
主題:
Original Paper
オンライン･アクセス:https://ncbi.nlm.nih.gov/pmc/articles/PMC7360660/
https://ncbi.nlm.nih.gov/pubmed/32562018
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00401-020-02176-0
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