Remarkably, these proteins not only functionally mediate direct interactions with various nucleic acids to control both RNA processing and gene expression, but their aggregation/fibrillation is pathologically characteristic of an increasing spectrum of human diseases including ALS, frontotemporal dementia (FTD), Alzheimer’s disease (AD), chronic traumatic encephalopathy, muscle regeneration/degeneration and multisystem proteinopathy (MSP)1–10. Here, ATAD1 is linked to frontotemporal dementia.