The constitutive activation (i.e., dephosphorylation) of TFEB and subsequent amplification of its transcriptional program, is the main oncogenic driver of the kidney abnormalities in a mouse model of BHD syndrome, while TFEB depletion rescues renal pathology and lethality in FLCN-knockout mice (Napolitano et al., 2020). Here, TFEB is linked to Birt-Hogg-Dubé syndrome.