Taken together, these alterations (among many others) help promote a state of endothelial dysfunction (ED) which, as initially stated, is the main characteristic of AT; it is characterized by a complex pathophysiology that is based on eNOS uncoupling and endothelial activation following abnormal gene expression from various inflammatory mediators as COX-2 is increased, vWF is released, and the downstream signaling via nuclear factor-kB (NF-kB) leads to the overexpression cytokines and adhesion molecules [12]. Here, VWF is linked to ataxia telangiectasia.