TM levels are increased in human CCM lesions and in the plasma of CCM patients, while, in mice, endothelial-specific deletion of KRIT1 or CCM3 results in KLF2/KLF4-mediated increased levels of vascular TM and endothelial protein C receptor (EPCR) [98]. This evidence concerns the gene KRIT1 and cerebral cavernous malformation.