Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

The exact molecular mechanisms driving FUS-mediated toxicity remain unclear. Here, the authors demonstrate that muscleblind (Mbl) is a novel modifier of FUS-associated ALS, with knockdown of endogenous Mbl suppressing neuromuscular junction defects and motor dysfunctions associated with FUS expressi...

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Autores principales:	Ian Casci, Karthik Krishnamurthy, Sukhleen Kour, Vadreenath Tripathy, Nandini Ramesh, Eric N. Anderson, Lara Marrone, Rogan A. Grant, Stacie Oliver, Lauren Gochenaur, Krishani Patel, Jared Sterneckert, Amanda M. Gleixner, Christopher J. Donnelly, Marc-David Ruepp, Antonella M. Sini, Emanuela Zuccaro, Maria Pennuto, Piera Pasinelli, Udai Bhan Pandey
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2019
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/808f528e987e4f6fbf0a7d25207ff6ed
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spelling	oai:doaj.org-article:808f528e987e4f6fbf0a7d25207ff6ed2021-12-02T15:36:22ZMuscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization10.1038/s41467-019-13383-z2041-1723https://doaj.org/article/808f528e987e4f6fbf0a7d25207ff6ed2019-12-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-13383-zhttps://doaj.org/toc/2041-1723The exact molecular mechanisms driving FUS-mediated toxicity remain unclear. Here, the authors demonstrate that muscleblind (Mbl) is a novel modifier of FUS-associated ALS, with knockdown of endogenous Mbl suppressing neuromuscular junction defects and motor dysfunctions associated with FUS expression in Drosophila, as well as restoring reduced SMN protein levels in mammalian neuronal and human iPSC-derived motor neurons.Ian CasciKarthik KrishnamurthySukhleen KourVadreenath TripathyNandini RameshEric N. AndersonLara MarroneRogan A. GrantStacie OliverLauren GochenaurKrishani PatelJared SterneckertAmanda M. GleixnerChristopher J. DonnellyMarc-David RueppAntonella M. SiniEmanuela ZuccaroMaria PennutoPiera PasinelliUdai Bhan PandeyNature PortfolioarticleScienceQENNature Communications, Vol 10, Iss 1, Pp 1-20 (2019)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Ian Casci Karthik Krishnamurthy Sukhleen Kour Vadreenath Tripathy Nandini Ramesh Eric N. Anderson Lara Marrone Rogan A. Grant Stacie Oliver Lauren Gochenaur Krishani Patel Jared Sterneckert Amanda M. Gleixner Christopher J. Donnelly Marc-David Ruepp Antonella M. Sini Emanuela Zuccaro Maria Pennuto Piera Pasinelli Udai Bhan Pandey Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
description	The exact molecular mechanisms driving FUS-mediated toxicity remain unclear. Here, the authors demonstrate that muscleblind (Mbl) is a novel modifier of FUS-associated ALS, with knockdown of endogenous Mbl suppressing neuromuscular junction defects and motor dysfunctions associated with FUS expression in Drosophila, as well as restoring reduced SMN protein levels in mammalian neuronal and human iPSC-derived motor neurons.
format	article
author	Ian Casci Karthik Krishnamurthy Sukhleen Kour Vadreenath Tripathy Nandini Ramesh Eric N. Anderson Lara Marrone Rogan A. Grant Stacie Oliver Lauren Gochenaur Krishani Patel Jared Sterneckert Amanda M. Gleixner Christopher J. Donnelly Marc-David Ruepp Antonella M. Sini Emanuela Zuccaro Maria Pennuto Piera Pasinelli Udai Bhan Pandey
author_facet	Ian Casci Karthik Krishnamurthy Sukhleen Kour Vadreenath Tripathy Nandini Ramesh Eric N. Anderson Lara Marrone Rogan A. Grant Stacie Oliver Lauren Gochenaur Krishani Patel Jared Sterneckert Amanda M. Gleixner Christopher J. Donnelly Marc-David Ruepp Antonella M. Sini Emanuela Zuccaro Maria Pennuto Piera Pasinelli Udai Bhan Pandey
author_sort	Ian Casci
title	Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
title_short	Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
title_full	Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
title_fullStr	Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
title_full_unstemmed	Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization
title_sort	muscleblind acts as a modifier of fus toxicity by modulating stress granule dynamics and smn localization
publisher	Nature Portfolio
publishDate	2019
url	https://doaj.org/article/808f528e987e4f6fbf0a7d25207ff6ed
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Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

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