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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Udgivet i:Sci Adv
Main Authors: 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
Format: Artigo
Sprog:Inglês
Udgivet: American Association for the Advancement of Science 2021
Fag:
Research Articles
Online adgang: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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