Among them, mutations in charged multivesicular body protein 2B (CHMP2B) are especially interesting, as they are highly pathogenic in FTD linked to chromosome 3 (FTD-3) [19] and also found in some ALS cases [4, 18] and patients with early-onset Alzheimer’s disease (AD) [9]. Here, CHMP2B is linked to frontotemporal dementia.