Article
M6A reduction relieves FUS-associated ALS granules.
Nature communications - 12 Jun 2024
Di Timoteo Gaia, Giuliani Andrea, Setti Adriano, Biagi Martina C, Lisi Michela, Santini Tiziana, Grandioso Alessia, Mariani Davide, Castagnetti Francesco, Perego Eleonora, Zappone Sabrina, Lattante Serena, Sabatelli Mario, Rotili Dante, Vicidomini Giuseppe, Bozzoni Irene
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease due to gradual motoneurons (MN) degeneration. Among the processes associated to ALS pathogenesis, there is the formation of cytoplasmic inclusions produced by aggregation of mutant proteins, among which the RNA binding protein FUS. Here we show that, in neuronal cells and in iPSC-derived MN expressing mutant FUS, such inclusions are...
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