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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Publicado en:Acta Neuropathol
Autores principales: 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
Formato: Artigo
Lenguaje:Inglês
Publicado: Springer Berlin Heidelberg 2020
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Original Paper
Acceso en línea: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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