Furthermore, sustained activation of AMPK may also result in nuclear accumulation of Nrf2 [102], an adaptive response to altered redox homeostasis that we have previously associated with loss of KRIT1 function in cellular models and surgical samples of CCM disease [22,23], and which we have now found to occur in the liver of KRIT1+/− mice as well. This evidence concerns the gene KRIT1 and cerebral cavernous malformation.