ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers.

CONCLUSIONS: We show that in fibroblasts of FUS P525L mutation carriers, FUS mislocalized to the cytoplasm where it redistributed into stress granules with likely a dose effect, i.e. a higher number of cells with granules, which persist longer, than in controls and ALS cases. These data represent an early molecular change occurring before ALS onset, suggesting a transient preaggregative state.
PMID: 29035885 [PubMed - as supplied by publisher]

Source: Neuro-Degenerative Diseases - October 17, 2017 Category: Neurology Authors: Lo Bello M, Di Fini F, Notaro A, Spataro R, Conforti FL, La Bella V Tags: Neurodegener Dis Source Type: research