Article
Mutations in microRNA processing genes in Wilms tumors derepress the IGF2 regulator PLAG1.
Genes & development - 1 Aug 2018
Chen Kenneth S, Stroup Emily K, Budhipramono Albert, Rakheja Dinesh, Nichols-Vinueza Diana, Xu Lin, Stuart Sarai H, Shukla Abhay A, Fraire Claudette, Mendell Joshua T, Amatruda James F
Abstract excerpt
Many childhood Wilms tumors are driven by mutations in the microRNA biogenesis machinery, but the mechanism by which these mutations drive tumorigenesis is unknown. Here we show that the transcription factor pleomorphic adenoma gene 1 (PLAG1) is a microRNA target gene that is overexpressed in Wilms tumors with mutations in microRNA processing genes. Wilms tumors can also overexpress PLAG1 through copy number...
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