BAG3 and myopathy: Although BAG3 is expressed in various tissues, it is particularly abundant in the bone and myocardium under mechanical strain.[14] BAG3 deficiency can lead to fulminant myopathy and early death in mice.[15] The clinical manifestations are rapid progressive weakness cardiomyopathy, axonal multiple neuropathy, severe respiratory insufficiency, and possible scoliosis in the first or second decade of life, which is consistent with the phenotype of our patient.