Pre-B-cell leukemias in Btk/Slp65-deficient mice arise independently of ongoing V(D)J recombination activity

Leukemia. 2011 Jan;25(1):48-56. doi: 10.1038/leu.2010.246. Epub 2010 Oct 29.

Abstract

The adapter protein Slp65 and Bruton's tyrosine kinase (Btk) are key components of the precursor-B (pre-B) cell receptor (pre-BCR) signaling pathway. Slp65-deficient mice spontaneously develop pre-B-cell leukemia, expressing high levels of the pre-BCR on their cell surface. As leukemic Slp65-deficient pre-B cells express the recombination activating genes (Rag)1 and Rag2, and manifest ongoing immunoglobulin (Ig) light-chain rearrangement, it has been hypothesized that deregulated recombinase activity contributes to malignant transformation. In this report, we investigated whether Rag-induced DNA damage is involved in oncogenic transformation of Slp65-deficient B cells. We employed Btk/Slp65 double-deficient mice carrying an autoreactive 3-83μδ BCR transgene. When developing B cells in their bone marrow express this BCR, the V(D)J recombination machinery will be activated, allowing for secondary Ig light-chain gene rearrangements to occur. This phenomenon, called receptor editing, will rescue autoreactive B cells from apoptosis. We observed that 3-83μδ transgenic Btk/Slp65 double-deficient mice developed B-cell leukemias expressing both the 3-83μδ BCR and the pre-BCR components λ5/VpreB. Importantly, such leukemias were found at similar frequencies in mice concomitantly deficient for Rag1 or the non-homologous end-joining factor DNA-PKcs. We therefore conclude that malignant transformation of Btk/Slp65 double-deficient pre-B cells is independent of deregulated V(D)J recombination activity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing / physiology*
  • Agammaglobulinaemia Tyrosine Kinase
  • Animals
  • Homeodomain Proteins / physiology
  • Immunoglobulin Joining Region / genetics
  • Immunoglobulin Light Chains / genetics
  • Immunoglobulin Variable Region / genetics
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Pre-B Cell Receptors / physiology
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / etiology*
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / immunology
  • Protein-Tyrosine Kinases / physiology*
  • Recombination, Genetic*

Substances

  • Adaptor Proteins, Signal Transducing
  • B cell linker protein
  • Homeodomain Proteins
  • Immunoglobulin Joining Region
  • Immunoglobulin Light Chains
  • Immunoglobulin Variable Region
  • Pre-B Cell Receptors
  • RAG-1 protein
  • Protein-Tyrosine Kinases
  • Agammaglobulinaemia Tyrosine Kinase
  • Btk protein, mouse