Treatment with oprozomib, ixazomib and EB1089 in myotubes from a dysferlinopathy patient promoted a trend towards increased expression of dysferlin and myogenin, but this low increase did not translate into higher sarcolemmal repair neither higher fusion index than that in untreated myotubes. Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.