FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation

FUsed in Sarcoma (FUS) is a multifunctional RNA binding protein (RBP). FUS mutations lead to its cytoplasmic mislocalization and cause the neurodegenerative disease amyotrophic lateral sclerosis (ALS). Here, we use mouse and human models with endogenous ALS-associated mutations to study the early co...

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Pubblicato in:Sci Adv
Autori principali: Birsa, Nicol, Ule, Agnieszka M., Garone, Maria Giovanna, Tsang, Brian, Mattedi, Francesca, Chong, P. Andrew, Humphrey, Jack, Jarvis, Seth, Pisiren, Melis, Wilkins, Oscar G., Nosella, Micheal L., Devoy, Anny, Bodo, Cristian, de la Fuente, Rafaela Fernandez, Fisher, Elizabeth M. C., Rosa, Alessandro, Viero, Gabriella, Forman-Kay, Julie D., Schiavo, Giampietro, Fratta, Pietro
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Association for the Advancement of Science 2021
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Research Articles
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC8294762/
https://ncbi.nlm.nih.gov/pubmed/34290090
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/sciadv.abf8660
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