Thus, MATR3 is among the family of genes including VCP, HNRNPA1, HNRNPA2B1 and SQSTM1 that cause “multisystem proteinopathy” associated with either one or a combination of ALS/frontotemporal dementia (FTD), VCPDM and Paget’s disease of bone [18]. Here, MATR3 is linked to frontotemporal dementia.