Additional file 14 of Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD

Additional file 14. Model of DPR-mediated inhibition of DNA DSB repair. In nucleoli, PR binds and stabilizes NPM1 pentamers by binding to the acidic loop of NPM1 in a similar way that endogenous arginine-rich proteins like ARF bind and stabilize NPM1. Since NPM1 facilitates DNA DSB repair in the nucleoplasm as a monomer, we hypothesize that PR inhibits DNA DSB repair, in part, by preventing the translocation of NPM1 from the nucleolus to the nucleoplasm.