The authors concluded that the myofibrillary muscle tissue phenotype of LGMD1D patients could be due to an inefficient maintenance of the sarcomeric structure or an increased accumulation of misfolded sarcomeric proteins caused by the impairment of the CASA system, although experimental evidence of a direct role of DNAJB6 in the CASA machinery has yet to be provided. Here, CSN1S1 is linked to Autosomal dominant limb-girdle muscular dystrophy type 1D.