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. Author manuscript; available in PMC: 2022 Jun 1.
Published in final edited form as: Urol Oncol. 2020 Apr 21;39(6):322–326. doi: 10.1016/j.urolonc.2020.03.016

Figure 2.

Figure 2.

Tumor suppressor regulation in renal cancer. The tumor suppressive activity of FLCN and Tsc2 is supported by the Hsp90 chaperone. FNIP1/2 and Tsc1 co-chaperones scaffold these Hsp90 clients, and together these complexes act to inhibit mTOR. Mutations in the Hsp90 clients FLCN and Tsc2 cause Birt-Hogg-Dubé (BHD) and Tuberous Sclerosis (TS) syndromes, respectively. The patient mutation FLCN-L460Qfsx25 cannot bind to FNIP1/2 and is degraded, though its expression is partially rescued by the Tsc1-Hsp90 chaperone complex. This compromises Tsc2 stability, leading to the clinical manifestation of angiomyolipoma in BHD patients.