Overall, these findings provided strong evidence that a reduction in the GSH:GSSG redox ratio caused by KRIT1 loss-of-function mutations results in enhanced S-glutathionylation of distinct structural and regulatory proteins, thus revealing a novel molecular signature in the stunning complexity of CCM disease and providing a novel framework for the identification of new disease biomarkers and therapeutic targets and the designing of specific and efficacious pharmacologic interventions. Here, KRIT1 is linked to cerebral cavernous malformation.