Dynamic evaluation of CYTO‐ID‐, LYSO‐ID‐ and/or Magic Red‐stained primary and immortalized plectin‐deficient myoblasts and EBS‐MD patient‐derived fibroblasts further substantiated that loss of plectin unequivocally provokes a dysfunction in the autophagosomal‐lysosomal turnover on the cellular level. The gene discussed is PLEC; the disease is Menkes disease.