TY  - JOUR
AU  - LaClair, Katherine D
AU  - Zhou, Qihui
AU  - Michaelsen, Meike
AU  - Wefers, Benedikt
AU  - Brill, Monika S
AU  - Janjic, Aleksandar
AU  - Rathkolb, Birgit
AU  - Farny, Daniel
AU  - Cygan, Mikolaj
AU  - de Angelis, Martin Hrabe
AU  - Wurst, Wolfgang
AU  - Neumann, Manuela
AU  - Enard, Wolfgang
AU  - Misgeld, Thomas
AU  - Arzberger, Thomas
AU  - Edbauer, Dieter
TI  - Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
JO  - Acta neuropathologica
VL  - 140
IS  - 2
SN  - 1432-0533
CY  - Heidelberg
PB  - Springer
M1  - DZNE-2020-01337
SP  - 121 - 142
PY  - 2020
AB  - 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
KW  - Amyotrophic Lateral Sclerosis: genetics
KW  - Amyotrophic Lateral Sclerosis: immunology
KW  - Amyotrophic Lateral Sclerosis: pathology
KW  - Animals
KW  - C9orf72 Protein: genetics
KW  - DNA Repeat Expansion: genetics
KW  - Disease Models, Animal
KW  - Interferons: biosynthesis
KW  - Mice
KW  - Mice, Transgenic
KW  - Nerve Degeneration: genetics
KW  - Nerve Degeneration: immunology
KW  - Nerve Degeneration: pathology
KW  - Neurons: pathology
LB  - PUB:(DE-HGF)16
C6  - pmid:32562018
C2  - pmc:PMC7360660
DO  - DOI:10.1007/s00401-020-02176-0
UR  - https://pub.dzne.de/record/153340
ER  -