Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm.

Journal article

Fused in sarcoma (FUS) belongs to the group of RNA-binding proteins implicated as underlying factors in amyotrophic lateral sclerosis (ALS) and certain other neurodegenerative diseases. Multiple FUS gene mutations have been linked to hereditary forms, and aggregation of FUS protein is believed to play an important role in pathogenesis of these diseases. In cultured cells, FUS variants with disease-associated amino acid substitutions or short deletions affecting nuclear localization signal (NL...

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Authors: Shelkovnikova, T; Robinson, H; Connor-Robson, N; Buchman, V

• Journal: Cell cycle (Georgetown, Tex.)
• Volume: 12
• Issue: 19
• Pages: 3194-3202
• Publication date: 2013-10-01
• DOI: 10.4161/cc.26241
• EISSN: 1551-4005
• ISSN: 1538-4101
• Language: English
• Keywords: Protein Binding; Humans; RNA; Recombinant Fusion Proteins; RNA-Binding Protein FUS; Mutation; Protein Structure, Tertiary; Cytoplasmic Granules; Cell Line, Tumor; Cytoplasm