Of note, mutations in DNAJB6 cause limb-girdle muscular dystrophy type 1D (LGMD1D) and potentially FTD (reviewed in ref. 83), and LGMD1D muscle fibers exhibit rimmed vacuoles and TDP-43- and ubiquitin-positive inclusions resembling those found in MSP1 (refs. 70,84). This evidence concerns the gene DNAJB6 and autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6).