Strikingly, the outcomes of these experiments showed clearly that treatment of KRIT1-deficient ECs with the antioxidant Tiron restored the levels of N1ICD in a dose-dependent manner (Figure 4A), demonstrating that the downregulation of Notch1 activation consequent to KRIT1 loss-of-function is indeed another redox-dependent phenomenon that may underlie the pathogenesis of CCM disease. This evidence concerns the gene KRIT1 and cerebral cavernous malformation.