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...

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Bibliografske podrobnosti
izdano v:Acta Neuropathol
Main Authors: 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
Format: Artigo
Jezik:Inglês
Izdano: Springer Berlin Heidelberg 2020
Teme:
Original Paper
Online dostop: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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