Although the FLCN-FNIP GAP activity toward RagC/D identifies this complex as a positive regulator of mTORC1, renal tumors from BHD patients containing FLCN germline mutations showed increased mTORC1 activity (Baba et al., 2008; Hasumi et al., 2009), as well as higher levels of phosphorylation of mTORC1 substrates (Khabibullin et al., 2014). The gene discussed is FLCN; the disease is kidney neoplasm.