PLEC and Menkes disease: Interestingly, the rodless plectin mice were viable and fertile and did not show any signs of skin blistering or muscular dystrophy, indicating that rodless plectin can functionally compensate for full-length plectin and that in EBS-MD patients, the overall rather low expression or complete loss of plectin rather than the absence of the rod domain is crucial for disease development [78].