Oncogene regulation. An oncogenic super-enhancer formed through somatic mutation of a noncoding intergenic element

Science. 2014 Dec 12;346(6215):1373-7. doi: 10.1126/science.1259037. Epub 2014 Nov 13.

Abstract

In certain human cancers, the expression of critical oncogenes is driven from large regulatory elements, called super-enhancers, that recruit much of the cell's transcriptional apparatus and are defined by extensive acetylation of histone H3 lysine 27 (H3K27ac). In a subset of T-cell acute lymphoblastic leukemia (T-ALL) cases, we found that heterozygous somatic mutations are acquired that introduce binding motifs for the MYB transcription factor in a precise noncoding site, which creates a super-enhancer upstream of the TAL1 oncogene. MYB binds to this new site and recruits its H3K27 acetylase-binding partner CBP, as well as core components of a major leukemogenic transcriptional complex that contains RUNX1, GATA-3, and TAL1 itself. Additionally, most endogenous super-enhancers found in T-ALL cells are occupied by MYB and CBP, which suggests a general role for MYB in super-enhancer initiation. Thus, this study identifies a genetic mechanism responsible for the generation of oncogenic super-enhancers in malignant cells.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Acetylation
  • Base Sequence
  • Basic Helix-Loop-Helix Transcription Factors / genetics*
  • Binding Sites
  • Cell Line, Tumor
  • DNA, Intergenic*
  • Enhancer Elements, Genetic*
  • Gene Expression Regulation, Neoplastic*
  • Histones / metabolism
  • Humans
  • INDEL Mutation*
  • Molecular Sequence Data
  • Mutation*
  • Oncogenes
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • Protein Interaction Domains and Motifs
  • Proto-Oncogene Proteins / genetics*
  • Proto-Oncogene Proteins c-myb / metabolism
  • T-Cell Acute Lymphocytic Leukemia Protein 1

Substances

  • Basic Helix-Loop-Helix Transcription Factors
  • DNA, Intergenic
  • Histones
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-myb
  • T-Cell Acute Lymphocytic Leukemia Protein 1
  • TAL1 protein, human