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Birt–Hogg–Dubé syndrome (BHD) is a human cancer disorder caused by mutations in the tumor suppressor gene  Folliculin  ( FLCN ) with unknown biological functions. Here, we show that the  Drosophila  homolog of FLCN, dFLCN (a.k.a.  dBHD ) localizes to the nucleolus and physically interacts with the 19S proteasomal ATPase, Rpt4, a nucleolar resident and known regulator of rRNA transcription. Downregulation of  dFLCN  resulted in an increase in nucleolar volume and upregulation of rRNA synthesis, whereas  dFLCN  overexpression reduced rRNA transcription and counteracted the effects of Rpt4 on rRNA production by preventing the association of Rpt4 with the rDNA locus. We further show that human FLCN exhibited evolutionarily conserved function and that Rpt4 knockdown inhibits the growth of FLCN-deficient human renal cancer cells in mouse xenografts. Our study suggests that FLCN functions as a tumor suppressor by negatively regulating rRNA synthesis.

The Birt–Hogg–Dubé tumor suppressor Folliculin negatively regulates ribosomal RNA synthesis