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Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

Amyotrophic lateral sclerosis (ALS) is the most frequent motor neuron disease. Cytoplasmic fused in sarcoma (FUS) aggregates are pathological hallmarks of FUS-ALS. Proper shuttling between the nucleus and cytoplasm is essential for physiological cell function. However, the initial event in the patho...

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Detalles Bibliográficos
Autores principales: Naumann, Marcel, Pal, Arun, Goswami, Anand, Lojewski, Xenia, Japtok, Julia, Vehlow, Anne, Naujock, Maximilian, Günther, René, Jin, Mengmeng, Stanslowsky, Nancy, Reinhardt, Peter, Sterneckert, Jared, Frickenhaus, Marie, Pan-Montojo, Francisco, Storkebaum, Erik, Poser, Ina, Freischmidt, Axel, Weishaupt, Jochen H., Holzmann, Karlheinz, Troost, Dirk, Ludolph, Albert C., Boeckers, Tobias M., Liebau, Stefan, Petri, Susanne, Cordes, Nils, Hyman, Anthony A., Wegner, Florian, Grill, Stephan W., Weis, Joachim, Storch, Alexander, Hermann, Andreas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5780468/
https://www.ncbi.nlm.nih.gov/pubmed/29362359
http://dx.doi.org/10.1038/s41467-017-02299-1