Evidence for KEAP1 succination came from the observation that there is striking upregulation of the nuclear factor (erythroid-derived 2)-like 2 (NRF2)-mediated antioxidant signaling pathway in our murine Fh1 deficient renal cyst model, mouse embryonic fibroblasts as well as human FH-deficient cells and tissues (Adam et al., 2011; Ooi et al., 2011). This evidence concerns the gene FLNB and cystic kidney disease.