To elucidate the pathogenetic mechanisms underlying the DNAJB6-related myopathy, animal models have been created showing that, indeed, conditional muscular expression of a DNAJB6 mutant in the mouse causes a LGMD1D myofibrillary muscle tissue phenotype. The gene discussed is DNAJB6; the disease is autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6).