ID

45807

Descripción

Principal Investigator: Adam Resnick, PhD, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, PA, USA MeSH: Glioma,Astrocytoma https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs001054 Pediatric low-grade gliomas (PLGGs) are the most common pediatric brain-tumor, with more than ten histologic subtypes recognized by the World Health Organization. We performed a genomic analysis of 230 PLGGs of which 73 had whole genome/RNA sequencing performed and show that MYB-QKI fusions define the seizure associated tumor, Angiocentric Glioma (AG). MYB-QKI fusions present in AGs contribute to tumorigenesis through three mechanisms: MYB activation by truncation, enhancer translocation driving aberrant MYB-QKI expression, and hemizygous loss of QKI, a tumor suppressor gene. Such interplay between three oncogenic mechanisms has diagnostic and therapeutic implications in AGs, and illustrates the functional complexity associated with rearrangements in cancer.

Link

dbGaP-study=phs001054

Palabras clave

  1. 23/6/23 23/6/23 - Chiara Middel
Titular de derechos de autor

Adam Resnick, PhD, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, PA, USA

Subido en

23 de junio de 2023

DOI

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Licencia

Creative Commons BY 4.0

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dbGaP phs001054 Genomic Characterization of Pediatric Low-Grade Gliomas

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