%0 Journal Article
%A LaClair, Katherine D
%A Zhou, Qihui
%A Michaelsen, Meike
%A Wefers, Benedikt
%A Brill, Monika S
%A Janjic, Aleksandar
%A Rathkolb, Birgit
%A Farny, Daniel
%A Cygan, Mikolaj
%A de Angelis, Martin Hrabe
%A Wurst, Wolfgang
%A Neumann, Manuela
%A Enard, Wolfgang
%A Misgeld, Thomas
%A Arzberger, Thomas
%A Edbauer, Dieter
%T Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
%J Acta neuropathologica
%V 140
%N 2
%@ 1432-0533
%C Heidelberg
%I Springer
%M DZNE-2020-01337
%P 121 - 142
%D 2020
%X Expansion of a (G4C2)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 vitro, but poly-GA is far more abundant in patients. To directly compare these in vivo, we created congenic poly-GA and poly-PR mice. 40
%K Amyotrophic Lateral Sclerosis: genetics
%K Amyotrophic Lateral Sclerosis: immunology
%K Amyotrophic Lateral Sclerosis: pathology
%K Animals
%K C9orf72 Protein: genetics
%K DNA Repeat Expansion: genetics
%K Disease Models, Animal
%K Interferons: biosynthesis
%K Mice
%K Mice, Transgenic
%K Nerve Degeneration: genetics
%K Nerve Degeneration: immunology
%K Nerve Degeneration: pathology
%K Neurons: pathology
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:32562018
%2 pmc:PMC7360660
%R 10.1007/s00401-020-02176-0
%U https://pub.dzne.de/record/153340