Article
Mutant ACVR1 Arrests Glial Cell Differentiation to Drive Tumorigenesis in Pediatric Gliomas.
Cancer cell - 16 Mar 2020
Fortin Jerome, Tian Ruxiao, Zarrabi Ida, Hill Graham, Williams Eleanor, Sanchez-Duffhues Gonzalo, Thorikay Midory, Ramachandran Parameswaran, Siddaway Robert, Wong Jong Fu, Wu Annette, Apuzzo Lorraine N, Haight Jillian, You-Ten Annick, Snow Bryan E, Wakeham Andrew, Goldhamer David J, Schramek Daniel, Bullock Alex N, Dijke Peter Ten, Hawkins Cynthia, Mak Tak W
Abstract excerpt
Diffuse intrinsic pontine gliomas (DIPGs) are aggressive pediatric brain tumors for which there is currently no effective treatment. Some of these tumors combine gain-of-function mutations in ACVR1, PIK3CA, and histone H3-encoding genes. The oncogenic mechanisms of action of ACVR1 mutations are currently unknown. Using mouse models, we demonstrate that Acvr1G328V arrests the differentiation of oligodendroglial...
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