We studied the pathogenic consequences of 3 previously reported BAG3 missense mutations, located in the second IPV-motif of BAG3 (Fig. 1a) which mediates the interaction with sHSP family members, and causing distinct phenotypes: Pro209Leu (early-onset dilated cardiomyopathy and/or severe distal myopathy24), Pro209Gln (late-onset distal myopathy25), Pro209Ser (late-onset CMT226). Here, BAG3 is linked to dilated cardiomyopathy.