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Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models

ALS is a devastating neurodegenerative disease primarily affecting motor neurons. Mutations in TDP-43 cause some forms of the disease, and cytoplasmic TDP-43 aggregates accumulate in degenerating neurons of most ALS patients. Thus, strategies aimed at targeting the toxicity of cytoplasmic TDP-43 agg...

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Detalles Bibliográficos
Autores principales: Armakola, Maria, Higgins, Matthew J., Figley, Matthew D., Barmada, Sami J., Scarborough, Emily A., Diaz, Zamia, Fang, Xiaodong, Shorter, James, Krogan, Nevan J., Finkbeiner, Steven, Farese, Robert V., Gitler, Aaron D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3510335/
https://www.ncbi.nlm.nih.gov/pubmed/23104007
http://dx.doi.org/10.1038/ng.2434