Transcriptional silencing of {gamma}-globin by BCL11A involves long-range interactions and cooperation with SOX6

Genes Dev. 2010 Apr 15;24(8):783-98. doi: 10.1101/gad.1897310.

Abstract

The developmental switch from human fetal (gamma) to adult (beta) hemoglobin represents a clinically important example of developmental gene regulation. The transcription factor BCL11A is a central mediator of gamma-globin silencing and hemoglobin switching. Here we determine chromatin occupancy of BCL11A at the human beta-globin locus and other genomic regions in vivo by high-resolution chromatin immunoprecipitation (ChIP)-chip analysis. BCL11A binds the upstream locus control region (LCR), epsilon-globin, and the intergenic regions between gamma-globin and delta-globin genes. A chromosome conformation capture (3C) assay shows that BCL11A reconfigures the beta-globin cluster by modulating chromosomal loop formation. We also show that BCL11A and the HMG-box-containing transcription factor SOX6 interact physically and functionally during erythroid maturation. BCL11A and SOX6 co-occupy the human beta-globin cluster along with GATA1, and cooperate in silencing gamma-globin transcription in adult human erythroid progenitors. These findings collectively demonstrate that transcriptional silencing of gamma-globin genes by BCL11A involves long-range interactions and cooperation with SOX6. Our findings provide insight into the mechanism of BCL11A action and new clues for the developmental gene regulatory programs that function at the beta-globin locus.

Publication types

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

MeSH terms

  • Adult
  • Animals
  • Carrier Proteins / metabolism*
  • Cell Line
  • Erythroid Cells / cytology
  • Erythroid Cells / metabolism*
  • Erythropoiesis / physiology
  • GATA1 Transcription Factor / metabolism
  • Gene Expression Regulation, Developmental*
  • Gene Silencing*
  • Histones / metabolism
  • Humans
  • Mice
  • Nuclear Proteins / metabolism*
  • Protein Binding
  • Protein Transport
  • Repressor Proteins
  • SOXD Transcription Factors / metabolism*
  • gamma-Globins / metabolism*

Substances

  • BCL11A protein, human
  • Carrier Proteins
  • GATA1 Transcription Factor
  • GATA1 protein, human
  • Histones
  • Nuclear Proteins
  • Repressor Proteins
  • SOX6 protein, human
  • SOXD Transcription Factors
  • gamma-Globins