Comprehensive evaluation of human-derived anti-poly-GA antibodies in cellular and animal models of C9orf72 disease

Hexanucleotide G(4)C(2) repeat expansions in the C9orf72 gene are the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Dipeptide repeat proteins (DPRs) generated by translation of repeat-containing RNAs show toxic effects in vivo as well as in vitro and are key...

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Autores principales: Jambeau, Melanie, Meyer, Kevin D., Hruska-Plochan, Marian, Tabet, Ricardos, Lee, Chao-Zong, Ray-Soni, Ananya, Aguilar, Corey, Savage, Kitty, Mishra, Nibha, Cavegn, Nicole, Borter, Petra, Lin, Chun-Chia, Jansen-West, Karen R., Jiang, Jie, Freyermuth, Fernande, Li, Nan, De Rossi, Pierre, Pérez-Berlanga, Manuela, Jiang, Xin, Daughrity, Lilian M., Pereira, João, Narayanan, Sarav, Gu, Yuanzheng, Dhokai, Shekhar, Dalkilic-Liddle, Isin, Maniecka, Zuzanna, Weber, Julien, Workman, Michael, McAlonis-Downes, Melissa, Berezovski, Eugene, Zhang, Yong-Jie, Berry, James, Wainger, Brian J., Kankel, Mark W., Rushe, Mia, Hock, Christoph, Nitsch, Roger M., Cleveland, Don W., Petrucelli, Leonard, Gendron, Tania F., Montrasio, Fabio, Grimm, Jan, Polymenidou, Magdalini, Lagier-Tourenne, Clotilde
Formato: Online Artículo Texto
Lenguaje:English
Publicado: National Academy of Sciences 2022
Materias:
Biological Sciences
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9894253/
https://www.ncbi.nlm.nih.gov/pubmed/36454749
http://dx.doi.org/10.1073/pnas.2123487119

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