The BCL11A transcription factor directly activates RAG gene expression and V(D)J recombination

Mol Cell Biol. 2013 May;33(9):1768-81. doi: 10.1128/MCB.00987-12. Epub 2013 Feb 25.

Abstract

Recombination-activating gene 1 protein (RAG1) and RAG2 are critical enzymes for initiating variable-diversity-joining (VDJ) segment recombination, an essential process for antigen receptor expression and lymphocyte development. The transcription factor BCL11A is required for B cell development, but its molecular function(s) in B cell fate specification and commitment is unknown. We show here that the major B cell isoform, BCL11A-XL, binds the RAG1 promoter and Erag enhancer to activate RAG1 and RAG2 transcription in pre-B cells. We employed BCL11A overexpression with recombination substrates in a cultured pre-B cell line as well as Cre recombinase-mediated Bcl11a(lox/lox) deletion in explanted murine pre-B cells to demonstrate direct consequences of BCL11A/RAG modulation on V(D)J recombination. We conclude that BCL11A is a critical component of a transcriptional network that regulates B cell fate by controlling V(D)J recombination.

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.
  • Retracted Publication

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Carrier Proteins / analysis
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism*
  • Cell Line
  • Cells, Cultured
  • DNA-Binding Proteins / genetics*
  • Gene Deletion
  • Homeodomain Proteins / genetics*
  • Humans
  • Mice
  • Mice, Inbred C57BL
  • Nuclear Proteins / analysis
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism*
  • Precursor Cells, B-Lymphoid / metabolism
  • Promoter Regions, Genetic
  • Protein Isoforms / analysis
  • Protein Isoforms / genetics
  • Protein Isoforms / metabolism
  • Repressor Proteins
  • Transcriptional Activation*
  • Up-Regulation
  • V(D)J Recombination*

Substances

  • BCL11A protein, human
  • Bcl11a protein, mouse
  • Carrier Proteins
  • DNA-Binding Proteins
  • Homeodomain Proteins
  • Nuclear Proteins
  • Protein Isoforms
  • Repressor Proteins
  • V(D)J recombination activating protein 2
  • RAG-1 protein