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Document type:
Article; Journal Article; Research Support, Non-U.S. Gov't 
Author(s):
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 
Title:
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS. 
Abstract:
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% of poly-PR mice were affected with ataxia and seizures, requiring euthan...    »
 
Journal title abbreviation:
Acta Neuropathol (Berl) 
Year:
2020 
Journal volume:
140 
Journal issue:
Pages contribution:
121-142 
Print-ISSN:
0001-6322 
TUM Institution:
Lehrstuhl für Zellbiologie des Nervensystems (Prof. Misgeld)