Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics.

ABSTRACT:

Background

Amyotrophic lateral sclerosis (ALS)-linked fused in sarcoma/translocated in liposarcoma (FUS/TLS or FUS) is concentrated within cytoplasmic stress granules under conditions of induced stress. Since only the mutants, but not the endogenous wild-type FUS, are associated with stress granules under most of the stress conditions reported to date, the relationship between FUS and stress granules represents a mutant-specific phenotype and thus may be of significance in mutant-induced pathogenesis. While the association of mutant-FUS with stress granules is well established, the effect of the mutant protein on stress granules has not been examined. Here we investigated the effect of mutant-FUS on stress granule formation and dynamics under conditions of oxidative stress.

Resu

SUBMITTER: Baron DM 

PROVIDER: S-EPMC3766239 | biostudies-literature | 2013 Aug

REPOSITORIES: biostudies-literature