Article
Cytoplasmic aggregation of mutant FUS causes multistep RNA splicing perturbations in the course of motor neuron pathology.
Nucleic acids research - 23 Jun 2023
Rezvykh Alexander P, Ustyugov Aleksey A, Chaprov Kirill D, Teterina Ekaterina V, Nebogatikov Vladimir O, Spasskaya Daria S, Evgen'ev Michael B, Morozov Alexey V, Funikov Sergei Yu
Abstract excerpt
Dysfunction of the RNA-binding protein (RBP) FUS implicated in RNA metabolism can cause amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. Mutations affecting FUS nuclear localization can drive RNA splicing defects and stimulate the formation of non-amyloid inclusions in affected neurons. However, the mechanism by which FUS mutations contribute to the development of ALS remains uncertain....
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