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Titel:

Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.

Dokumenttyp:
Journal Article; Article
Autor(en):
Schludi, Martin H; Becker, Lore; Garrett, Lillian; Gendron, Tania F; Zhou, Qihui; Schreiber, Franziska; Popper, Bastian; Dimou, Leda; Strom, Tim M; Winkelmann, Juliane; von Thaden, Anne; Rentzsch, Kristin; May, Stephanie; Michaelsen, Meike; Schwenk, Benjamin M; Tan, Jing; Schoser, Benedikt; Dieterich, Marianne; Petrucelli, Leonard; Hölter, Sabine M; Wurst, Wolfgang; Fuchs, Helmut; Gailus-Durner, Valerie; de Angelis, Martin Hrabe; Klopstock, Thomas; Arzberger, Thomas; Edbauer, Dieter
Abstract:
Translation of the expanded (ggggcc)n repeat in C9orf72 patients with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) causes abundant poly-GA inclusions. To elucidate their role in pathogenesis, we generated transgenic mice expressing codon-modified (GA)149 conjugated with cyan fluorescent protein (CFP). Transgenic mice progressively developed poly-GA inclusions predominantly in motoneurons and interneurons of the spinal cord and brain stem and in deep cerebellar nuclei. Po...     »
Zeitschriftentitel:
Acta Neuropathol (Berl)
Jahr:
2017
Band / Volume:
134
Heft / Issue:
2
Seitenangaben Beitrag:
241-254
Sprache:
eng
Volltext / DOI:
doi:10.1007/s00401-017-1711-0
PubMed:
http://view.ncbi.nlm.nih.gov/pubmed/28409281
Print-ISSN:
0001-6322
TUM Einrichtung:
Institut für Humangenetik; Lehrstuhl für Neurogenetik (Prof. Winkelmann)
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