Heterozygous mutations in the gene encoding fumarate hydroatase (FH) have been reported to be associated with tumor formation, while high levels of fumarate accumulate and succinylation of protein can readily be detect in both FH-deficient cells and hereditary leiomyomatosis and renal cell carcinoma (HLRCC) (Tomlinson et al., 2002; Bardella et al., 2011; Mitsuishi et al., 2012). The gene discussed is FH; the disease is hereditary clear cell renal cell carcinoma.