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

Autor: 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
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Communications Biology, Vol 4, Iss 1, Pp 1-17 (2021)
Druh dokumentu: article
ISSN: 2399-3642
DOI: 10.1038/s42003-021-02538-8
Popis: 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.
Databáze: Directory of Open Access Journals
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