Journal
NATURE GENETICS
Volume 48, Issue 3, Pages 273-282Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/ng.3500
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Funding
- A Kids' Brain Tumor Cure Foundation Pediatric Low-Grade Astrocytoma Foundation
- US National Institutes of Health [R01NS085336, PO1CA142536]
- Voices Against Brain Cancer
- Children's Brain Tumor Foundation
- Stop and Shop Pediatric Brain Tumor Program
- Path to Cure Foundation
- St. Baldricks Foundation
- American Brain Tumor Association
- Team Jack Foundation
- Andrysiak Fund
- Broad Institute Scientific Projects to Accelerate Research and Collaboration (SPARC) grant
- Jared Branfman Sunflowers for Life Fund for Pediatric Brain and Spinal Cancer Research
- Sontag Foundation
- Nuovo-Soldati Foundation
- Philippe Foundation
- Fondation Etoile de Martin
- Damon Runyon-Sohn Pediatric Fellowship Award
- Hyundai Scholar Grant
- Bear Necessities Pediatric Cancer Foundation
- Rally Foundation for Childhood Cancer Research
- National Institute of Neurological Disorders and Stroke (NINDS) [K08NS087118]
- Pediatric Brain Tumor Foundation
- Thea's Star of Hope
- Hungarian Brain Research Program [KTIA_13_NAP-A-V/3]
- Janos Bolyai Scholarship of the Hungarian Academy of Sciences
- NINDS [1R01NS091620]
- Nancy and Stephen Grand Philanthropic Fund
- Pediatric Low-Grade Astrocytoma Foundation
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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.
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