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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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
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Publicado: Nature Portfolio 2020
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Acceso en línea:https://doaj.org/article/29a7f401a44547198252c6ea20fa553a
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spelling oai:doaj.org-article:29a7f401a44547198252c6ea20fa553a2021-12-02T13:24:34ZAberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis10.1038/s41467-020-20191-32041-1723https://doaj.org/article/29a7f401a44547198252c6ea20fa553a2020-12-01T00:00:00Zhttps://doi.org/10.1038/s41467-020-20191-3https://doaj.org/toc/2041-1723Mutations 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.Daniel JutziSébastien CampagneRalf SchmidtStefan ReberJonas MechtersheimerFoivos GypasChristoph SchweingruberMartino ColomboChristine von SchroetterFionna E. LoughlinAnny DevoyEva HedlundMihaela ZavolanFrédéric H.-T. AllainMarc-David RueppNature PortfolioarticleScienceQENNature Communications, Vol 11, Iss 1, Pp 1-14 (2020)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
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
Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
description 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.
format article
author 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
author_facet 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
author_sort Daniel Jutzi
title Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
title_short Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
title_full Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
title_fullStr Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
title_full_unstemmed Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis
title_sort aberrant interaction of fus with the u1 snrna provides a molecular mechanism of fus induced amyotrophic lateral sclerosis
publisher Nature Portfolio
publishDate 2020
url https://doaj.org/article/29a7f401a44547198252c6ea20fa553a
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