As plectin is abundantly expressed in these cells [7] and blood filled blisters have been observed in plectin-null mice [13] as well as in EBS-MD patients, an endothelium-restricted KO mouse, VE-Cre/cKO (plecloxP/loxP:Cdh5-Cre), was generated to examine the function of plectin in this stress-prone system [31]. The gene discussed is PLEC; the disease is Menkes disease.