On the other hand, inactivating mutation of FH (an enzyme involved in the mitochondrial tricarboxylic acid cycle) causes Nrf2-dependent activation of antioxidant pathways in patients with inherited type 2 pRCC (pRCC2) or hereditary leiomyomatosis and renal cell carcinoma (HLRCC). Here, NFE2L2 is linked to renal cell carcinoma.