Article
Recurrent ZC3H18 mutations stabilize oncogenic endogenous retroviral RNA.
Cell reports - 28 Jul 2026
Tanu Tanzina, Cox Anna M, Karlow Jennifer A, Sharma Priyanka, Sun Kaiwen, He Xueyang, Babu Swathy, Roelofs Madeline K, Jeon Ethan, Chen Jiajia, Wu Constance, Brown Jared, Barrientos Nelson B, McCallion Andrew S, Brown Kevin M, Chanock Stephen J, Liu David, Zhang Tongwu, Burns Kathleen H, Boutz Paul L, Insco Megan L
Abstract excerpt
Endogenous retroviral (ERV) RNA is highly expressed in cancer, suggesting a selective advantage. We identify recurrent truncating mutations in ZC3H18 (Z18), a nuclear RNA surveillance factor lost in 30% of all cancers. We show that Z18 truncating mutations are oncogenic and that Z18 plays an evolutionarily conserved role in the nuclear surveillance of oncogenic ERV RNA. In zebrafish, Z18 truncation accelerates...
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