ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

Mutations in the FUS gene cause amyotrophic lateral sclerosis (ALS-FUS). Mutant FUS is known to confer cytoplasmic gain of function but its effects in the nucleus are less understood. FUS is an essential component of paraspeckles, subnuclear bodies assembled on a lncRNA NEAT1. Paraspeckles may play...

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Publicat a:Acta Neuropathol Commun
Autors principals: An, Haiyan, Skelt, Lucy, Notaro, Antonietta, Highley, J. Robin, Fox, Archa H., La Bella, Vincenzo, Buchman, Vladimir L., Shelkovnikova, Tatyana A.
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2019
Matèries:
Research
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6330737/
https://ncbi.nlm.nih.gov/pubmed/30642400
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40478-019-0658-x
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