TDP-43-positive rimmed vacuole myopathies [31] include sporadic inclusion body myositis (sIBM) [10], hereditary inclusion body myopathy with early-onset Paget disease and FTD (caused by heterozygous mutations in VCP) [41, 60, 61], inclusion body myopathy associated with multisystem proteinopathy (caused by heterozygous mutations in HNRNPA2B1 and HNRNPA1) [28], distal myopathy with rimmed vacuoles (caused by bi-allelic mutations in GNE) [19] and oculopharyngeal muscular dystrophies (caused by heterozygous mutations in PABPN1, HNRNPA2B1 and HNRNPA1) [6, 9, 29]. Here, HNRNPA2B1 is linked to frontotemporal dementia.