Through advances in cryo-electron microscopy (cryo-EM), homotypic aggregates of the C-Terminal domain of TMEM106B (TMEM CT) were discovered as a previously unidentified cytosolic proteinopathy in the brains of FTLD, Alzheimer’s disease, progressive supranuclear palsy (PSP), and dementia with Lewy bodies (DLB) patients. Here, TMEM106B is linked to Classical progressive supranuclear palsy.