The protein aggregation myopathy Limb-Girdle Muscular Dystrophy 1D (LGMD1D) is caused by mutations of amino acids Phe89 or Phe93 of DNAJB6, a co-chaperone of the HSP70 anti-aggregation protein. Here, DNAJB6 is linked to autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6).