Increasing protein expression or activity of NIRs, either pharmacologically or by using engineered potentiated variants, may be used therapeutically to reverse pathological phase transition of ALS/FTD-related RBPs and other FG-Nup-associated disease proteins, and ultimately alleviate neurodegeneration. A recent cryo-EM study has shown that while all FET proteins formed insoluble aggregates with TNPO1 in the brain tissue of four FTLD-FUS cases, only TAF15 was observed to form filaments [320]. Here, FUS is linked to frontotemporal dementia.