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

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...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2017
Materias:
Original Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5508040/
https://www.ncbi.nlm.nih.gov/pubmed/28409281
http://dx.doi.org/10.1007/s00401-017-1711-0

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