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MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism.
Bandopadhayay, Pratiti; Ramkissoon, Lori A; Jain, Payal; Bergthold, Guillaume; Wala, Jeremiah; Zeid, Rhamy; Schumacher, Steven E; Urbanski, Laura; O'Rourke, Ryan; Gibson, William J; Pelton, Kristine; Ramkissoon, Shakti H; Han, Harry J; Zhu, Yuankun; Choudhari, Namrata; Silva, Amanda; Boucher, Katie; Henn, Rosemary E; Kang, Yun Jee; Knoff, David; Paolella, Brenton R; Gladden-Young, Adrianne; Varlet, Pascale; Pages, Melanie; Horowitz, Peleg M; Federation, Alexander; Malkin, Hayley; Tracy, Adam A; Seepo, Sara; Ducar, Matthew; Van Hummelen, Paul; Santi, Mariarita; Buccoliero, Anna Maria; Scagnet, Mirko; Bowers, Daniel C; Giannini, Caterina; Puget, Stephanie; Hawkins, Cynthia; Tabori, Uri; Klekner, Almos; Bognar, Laszlo; Burger, Peter C; Eberhart, Charles; Rodriguez, Fausto J; Hill, D Ashley; Mueller, Sabine; Haas-Kogan, Daphne A; Phillips, Joanna J; Santagata, Sandro; Stiles, Charles D.
Afiliação
  • Bandopadhayay P; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Ramkissoon LA; Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, Massachusetts, USA.
  • Jain P; Broad Institute, Cambridge, Massachusetts, USA.
  • Bergthold G; Harvard Medical School, Boston, Massachusetts, USA.
  • Wala J; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Zeid R; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Schumacher SE; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Urbanski L; Cell and Molecular Biology Graduate Group, Gene Therapy and Vaccines Program, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • O'Rourke R; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Gibson WJ; Department de Cancerologie de l'Enfant et de l'Adolescent et Unité Mixte de Recherche du Centre National de la Recherche Scientifique 8203 'Vectorologie et Nouvelles Therapeutiques du Cancer', Gustave Roussy, Université Paris XI Sud, Villejuif, France.
  • Pelton K; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Ramkissoon SH; Broad Institute, Cambridge, Massachusetts, USA.
  • Han HJ; Harvard Medical School, Boston, Massachusetts, USA.
  • Zhu Y; Harvard Medical School, Boston, Massachusetts, USA.
  • Choudhari N; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Silva A; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Boucher K; Broad Institute, Cambridge, Massachusetts, USA.
  • Henn RE; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Kang YJ; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Knoff D; Broad Institute, Cambridge, Massachusetts, USA.
  • Paolella BR; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Gladden-Young A; Broad Institute, Cambridge, Massachusetts, USA.
  • Varlet P; Harvard Medical School, Boston, Massachusetts, USA.
  • Pages M; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Horowitz PM; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Federation A; Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
  • Malkin H; Department of Pathology, Boston Children's Hospital, Boston, Massachusetts, USA.
  • Tracy AA; Department of Pathology, Harvard Medical School, Boston, Massachusetts, USA.
  • Seepo S; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Ducar M; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Van Hummelen P; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Santi M; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Buccoliero AM; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Scagnet M; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Bowers DC; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Giannini C; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Puget S; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Hawkins C; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Tabori U; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Klekner A; Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
  • Bognar L; Department of Neurosurgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
  • Burger PC; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Eberhart C; Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Rodriguez FJ; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
  • Hill DA; Broad Institute, Cambridge, Massachusetts, USA.
  • Mueller S; Harvard Medical School, Boston, Massachusetts, USA.
  • Haas-Kogan DA; Broad Technology Laboratories, Broad Institute, Cambridge, Massachusetts, USA.
  • Phillips JJ; Laboratoire de Neuropathologie, Hopital Sainte-Anne, Université Paris V Descartes, Paris, France.
  • Santagata S; Laboratoire de Neuropathologie, Hopital Sainte-Anne, Université Paris V Descartes, Paris, France.
  • Stiles CD; Department of Cancer Biology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
Nat Genet ; 48(3): 273-82, 2016 Mar.
Article em En | MEDLINE | ID: mdl-26829751
ABSTRACT
Angiocentric 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 that 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. To our knowledge, this represents the first example of a single driver rearrangement simultaneously transforming cells via three genetic and epigenetic mechanisms in a tumor.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Proteínas de Fusão Oncogênica / Proteínas de Ligação a RNA / Proteínas Oncogênicas v-myb / Glioma Tipo de estudo: Systematic_reviews Limite: Child / Humans Idioma: En Revista: Nat Genet Assunto da revista: GENETICA MEDICA Ano de publicação: 2016 Tipo de documento: Article País de afiliação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Proteínas de Fusão Oncogênica / Proteínas de Ligação a RNA / Proteínas Oncogênicas v-myb / Glioma Tipo de estudo: Systematic_reviews Limite: Child / Humans Idioma: En Revista: Nat Genet Assunto da revista: GENETICA MEDICA Ano de publicação: 2016 Tipo de documento: Article País de afiliação: Estados Unidos