Article
Mislocalised FUS mutants stall spliceosomal snRNPs in the cytoplasm.
Neurobiology of disease - 1 Jul 2013
Gerbino Valeria, Carrì Maria Teresa, Cozzolino Mauro, Achsel Tilmann
Abstract excerpt
Genes encoding RNA-binding proteins have frequently been implicated in various motor neuron diseases, but the particular step in RNA metabolism that is vulnerable in motor neurons remains unknown. FUS, a nuclear protein, forms cytoplasmic aggregates in cells affected by amyotrophic lateral sclerosis (ALS), and mutations disturbing the nuclear import of FUS cause the disease. It is extremely likely that the...
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