ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity

Maria Giovanna Garone et al. use iPSC and mouse models to evaluate a mechanistic link between aberrant axonal phenotypes in ALS and the alteration of a cross-regulatory circuitry involving three RNA binding proteins: FUS, HuD and FMRP. Their results suggest NRN1 as a potential therapeutic target for...

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Autores principales: Maria Giovanna Garone, Nicol Birsa, Maria Rosito, Federico Salaris, Michela Mochi, Valeria de Turris, Remya R. Nair, Thomas J. Cunningham, Elizabeth M. C. Fisher, Mariangela Morlando, Pietro Fratta, Alessandro Rosa
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Publicado: Nature Portfolio 2021
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Acceso en línea:https://doaj.org/article/4355909082b145b69e7ada11d6280eeb
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spelling oai:doaj.org-article:4355909082b145b69e7ada11d6280eeb2021-12-02T16:38:23ZALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity10.1038/s42003-021-02538-82399-3642https://doaj.org/article/4355909082b145b69e7ada11d6280eeb2021-09-01T00:00:00Zhttps://doi.org/10.1038/s42003-021-02538-8https://doaj.org/toc/2399-3642Maria Giovanna Garone et al. use iPSC and mouse models to evaluate a mechanistic link between aberrant axonal phenotypes in ALS and the alteration of a cross-regulatory circuitry involving three RNA binding proteins: FUS, HuD and FMRP. Their results suggest NRN1 as a potential therapeutic target for ALS and provide further insight into the pathogenesis of this critical disorder.Maria Giovanna GaroneNicol BirsaMaria RositoFederico SalarisMichela MochiValeria de TurrisRemya R. NairThomas J. CunninghamElizabeth M. C. FisherMariangela MorlandoPietro FrattaAlessandro RosaNature PortfolioarticleBiology (General)QH301-705.5ENCommunications Biology, Vol 4, Iss 1, Pp 1-17 (2021)
institution DOAJ
collection DOAJ
language EN
topic Biology (General)
QH301-705.5
spellingShingle Biology (General)
QH301-705.5
Maria Giovanna Garone
Nicol Birsa
Maria Rosito
Federico Salaris
Michela Mochi
Valeria de Turris
Remya R. Nair
Thomas J. Cunningham
Elizabeth M. C. Fisher
Mariangela Morlando
Pietro Fratta
Alessandro Rosa
ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
description Maria Giovanna Garone et al. use iPSC and mouse models to evaluate a mechanistic link between aberrant axonal phenotypes in ALS and the alteration of a cross-regulatory circuitry involving three RNA binding proteins: FUS, HuD and FMRP. Their results suggest NRN1 as a potential therapeutic target for ALS and provide further insight into the pathogenesis of this critical disorder.
format article
author Maria Giovanna Garone
Nicol Birsa
Maria Rosito
Federico Salaris
Michela Mochi
Valeria de Turris
Remya R. Nair
Thomas J. Cunningham
Elizabeth M. C. Fisher
Mariangela Morlando
Pietro Fratta
Alessandro Rosa
author_facet Maria Giovanna Garone
Nicol Birsa
Maria Rosito
Federico Salaris
Michela Mochi
Valeria de Turris
Remya R. Nair
Thomas J. Cunningham
Elizabeth M. C. Fisher
Mariangela Morlando
Pietro Fratta
Alessandro Rosa
author_sort Maria Giovanna Garone
title ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
title_short ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
title_full ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
title_fullStr ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
title_full_unstemmed ALS-related FUS mutations alter axon growth in motoneurons and affect HuD/ELAVL4 and FMRP activity
title_sort als-related fus mutations alter axon growth in motoneurons and affect hud/elavl4 and fmrp activity
publisher Nature Portfolio
publishDate 2021
url https://doaj.org/article/4355909082b145b69e7ada11d6280eeb
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