Accumulated evidence points to Krev interaction trapped protein 1 (KRIT1) as a major regulator of endothelial cell homeostasis and function and as a key determinant of the pathogenesis of Cerebral Cavernous Malformation (CCM), a major cerebrovascular disease of proven genetic origin characterized by the formation of clusters of abnormally dilated and leaky blood capillaries, which predispose to seizures, neurological deficits, and intracerebral hemorrhage (ICH) [4]. This evidence concerns the gene KRIT1 and famililal cerebral cavernous malformations.