Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis

Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS). Here the authors characterize FUS-binding to U1 snRNP and show that ALS-associated FUS aberrantly contacts U1 snRNA which interferes with its biogenesis pathway.

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Detalles Bibliográficos
Autores principales: Daniel Jutzi, Sébastien Campagne, Ralf Schmidt, Stefan Reber, Jonas Mechtersheimer, Foivos Gypas, Christoph Schweingruber, Martino Colombo, Christine von Schroetter, Fionna E. Loughlin, Anny Devoy, Eva Hedlund, Mihaela Zavolan, Frédéric H.-T. Allain, Marc-David Ruepp
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2020
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Acceso en línea:https://doaj.org/article/29a7f401a44547198252c6ea20fa553a
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Sumario:Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS). Here the authors characterize FUS-binding to U1 snRNP and show that ALS-associated FUS aberrantly contacts U1 snRNA which interferes with its biogenesis pathway.