Thus, dysregulation of FH and SDHB can contribute to HIF-1α-mediated pseudo-hypoxia driving RCC tumorigenesis through substrate inhibition of HIF-1α prolyl hydroxylation [Pollard et al., 2005] and, more recently, the identification of TCEB1 (MIM #600788) (encoding Elongin C) mutations in sporadic RCC [Sato et al., 2013]. This evidence concerns the gene ELOC and renal cell carcinoma.