Succination of the ubiquitin E3 ligase, KEAP1, is also seen in HLRCC leading to augmented levels of the anti-oxidant protein NRF2, thus allowing HLRCC tumors to ward off the deleterious effects from increased oxidative stress inherently found with mitochondrial dysfunction and tumorigenesis [140,141,143]. Here, KEAP1 is linked to hereditary leiomyomatosis and renal cell cancer.