This might not be unexpected given the number of other genes (including VCP, SQSTM1, HNRNPA1, HNRNPA2B1 and MATR3), in which mutations cause clinical syndromes, referred to as multisystem proteinopathies, with ALS, FTD, inclusion body myopathy and bone disease each showing variable penetrance [29]. The gene discussed is MATR3; the disease is frontotemporal dementia.