Bandopadhayay, PratitiRamkissoon, Lori A.Jain, PayalBergthold, GuillaumeWala, JeremiahZeid, RhamySchumacher, Steven E.Urbanski, LauraO’Rourke, RyanGibson, WilliamPelton, KristineRamkissoon, Shakti H.Han, Harry J.Zhu, YuankunChoudhari, NamrataSilva, AmandaBoucher, KatieHenn, Rosemary E.Kang, Yun JeeKnoff, DavidPaolella, Brenton R.Gladden-Young, AdrianneVarlet, PascalePages, MelanieHorowitz, Peleg M.Federation, AlexanderMalkin, HayleyTracy, AdamSeepo, SaraDucar, MatthewHummelen, Paul VanSanti, MariaritaBuccoliero, Anna MariaScagnet, MirkoBowers, Daniel C.Giannini, CaterinaPuget, StephanieHawkins, CynthiaTabori, UriKlekner, AlmosBognar, LaszloBurger, Peter C.Eberhart, CharlesRodriguez, Fausto J.Hill, D. AshleyMueller, SabineHaas-Kogan, DaphnePhillips, Joanna J.Santagata, SandroStiles, Charles D.Bradner, James EJabado, NadaGoren, AlonGrill, JacquesLigon, AzraGoumnerova, LilianaWaanders, Angela J.Storm, Phillip B.Kieran, Mark W.Ligon, KeithBeroukhim, RameenResnick, Adam C.2016-10-112016Bandopadhayay, P., L. A. Ramkissoon, P. Jain, G. Bergthold, J. Wala, R. Zeid, S. E. Schumacher, et al. 2016. “MYB-QKI rearrangements in Angiocentric Glioma drive tumorigenicity through a tripartite mechanism.” Nature genetics 48 (3): 273-282. doi:10.1038/ng.3500. http://dx.doi.org/10.1038/ng.3500.1061-4036http://nrs.harvard.edu/urn-3:HUL.InstRepos:29002633Angiocentric gliomas are pediatric low-grade gliomas (PLGGs) without known recurrent genetic drivers. We performed genomic analysis of new and published data from 249 PLGGs including 19 Angiocentric Gliomas. We identified MYB-QKI fusions as a specific and single candidate driver event in Angiocentric Gliomas. In vitro and in vivo functional studies show MYB-QKI rearrangements promote tumorigenesis through three mechanisms: MYB activation by truncation, enhancer translocation driving aberrant MYB-QKI expression, and hemizygous loss of the tumor suppressor QKI. This represents the first example of a single driver rearrangement simultaneously transforming cells via three genetic and epigenetic mechanisms in a tumor.en-USJournal Article2016-10-1110.1038/ng.3500