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C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice

The expansion of a hexanucleotide (GGGGCC) repeat in C9ORF72 is the most common cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Both the function of C9ORF72 and the mechanism by which the repeat expansion drives neuropathology are unknown. To examine whether C9ORF72 h...

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Detalles Bibliográficos
Autores principales: Atanasio, Amanda, Decman, Vilma, White, Derek, Ramos, Meg, Ikiz, Burcin, Lee, Hoi-Ching, Siao, Chia-Jen, Brydges, Susannah, LaRosa, Elizabeth, Bai, Yu, Fury, Wen, Burfeind, Patricia, Zamfirova, Ralica, Warshaw, Gregg, Orengo, Jamie, Oyejide, Adelekan, Fralish, Michael, Auerbach, Wojtek, Poueymirou, William, Freudenberg, Jan, Gong, Guochun, Zambrowicz, Brian, Valenzuela, David, Yancopoulos, George, Murphy, Andrew, Thurston, Gavin, Lai, Ka-Man Venus
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4793236/
https://www.ncbi.nlm.nih.gov/pubmed/26979938
http://dx.doi.org/10.1038/srep23204