Overexpression of Tfe3 in mice led to the formation of kidney cysts and cancer while the genetic depletion of Tfeb rescued the phenotype of mice with kidney-specific knockout of Flcn. Therefore, it is likely that the activation of TFEB is the main driver of the kidney pathology in BHD syndrome [86]. This evidence concerns the gene TFEB and Birt-Hogg-Dubé syndrome.