Despite the role of FLCN as a positive modulator of mTORC1 activity, mTORC1 is hyperactivated in renal tumors from BHD patients or FLCN knockout mouse models (Baba et al., 2008; Chen et al., 2008; Hasumi et al., 2009; Hudon et al., 2010). Here, FLCN is linked to Birt-Hogg-Dube syndrome.