The major genes are VHL (OMIM * 608537), FH (OMIM * 136850), MET (OMIM * 164860), and FLCN (OMIM * 607273), involved, respectively, in Von Hippel–Lindau (VHL) disease, hereditary leiomyomatosis and renal cell carcinoma (HLRCC), hereditary papillary renal cell carcinoma, and Birt–Hogg–Dubé (BHD) syndromes. The gene discussed is FLCN; the disease is Birt-Hogg-Dubé syndrome.