Thus, germline mutations in the VHL (MIM #608537) tumor suppressor gene (TSG) cause the familial cancer syndrome von Hippel–Lindau disease (MIM #193300) and somatic VHL inactivation is found in at least 80% of sporadic clear cell RCC [Banks et al., 2006; Foster et al., 1994; Gnarra et al., 1994; Latif et al., 1993; Maher et al., 1991]. Here, VHL is linked to von Hippel-Lindau disease.