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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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association for the Advancement of Science 2021
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8294762/
https://www.ncbi.nlm.nih.gov/pubmed/34290090
http://dx.doi.org/10.1126/sciadv.abf8660

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