sentences:  "FLCN"-deficient BHD associated renal tumors and tumors that develop in mouse models with "Flcn" inactivation were found to have elevated expression of transmembrane glycoprotein NMB (GPNMB), a transcriptional target of TFE3.Subsequently, FLCN was shown to regulate TFE3 activity by sequestering TFE3 in the cytoplasm where it is transcriptionally inactive; however, loss of FLCN expression results in localization of TFE3 to the nucleus driving transcriptional activation of its target genes including GPNMB.Another study investigating genes required for mouse embryonic stem cell (ESC) progression from pluripotency to cell lineage differentiation revealed that Flcn in complex with Fnip1/2 was necessary for ESC exit from pluripotency through cytoplasmic sequestering of Tfe3, thereby abrogating expression of its gene target, "estrogen-related receptor beta"("Esrrb"), the core pluripotency factor.