Considering the requirement for p97 and DNAJB6 during aggresome clearance reported here, it is tempting to speculate that failure of mutant p97 and DNAJB6 to efficiently clear aggresomes contributes to the pathogenesis of LGMD1D and MSP1. This evidence concerns the gene VCP and autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6).