Given the frequent observation of mTORC1 activation in the genetically engineered FLCN or FNIP-null mouse models [5–7, 9, 24, 27] as well as in patients with BHD [51], our results provide further support for the use of mTOR inhibitors for patients with BHD syndrome, which has been evaluated in both in vitro [52] and in vivo models [5, 7, 53] and is currently being evaluated in a clinical trial (NCT02504892). The gene discussed is MTOR; the disease is Birt-Hogg-Dubé syndrome.