Article
Inactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localization.
PloS one - 29 Dec 2010
Hong Seung-Beom, Oh HyoungBin, Valera Vladimir A, Baba Masaya, Schmidt Laura S, Linehan W Marston
Abstract excerpt
BACKGROUND: Germline mutations in a tumor suppressor gene FLCN lead to development of fibrofolliculomas, lung cysts and renal cell carcinoma (RCC) in Birt-Hogg-Dubé syndrome. TFE3 is a member of the MiTF/TFE transcription factor family and Xp11.2 translocations found in sporadic RCC involving TFE3 result in gene fusions and overexpression of chimeric fusion proteins that retain the C-terminal DNA binding domain...
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