FUS and frontotemporal dementia: A feature of degenerating neurons in various FUS proteinopathies, including ALS and frontotemporal dementia (FTD), is the depletion of FUS from the nucleus and the accumulation of FUS in cytoplasmic inclusions.10 Potentiated Hsp104 variants can mitigate cytoplasmic FUS aggregation and toxicity in yeast,29 but their activity in human cells has not been assessed.