Rearrangement of antigen receptor genes is defective in mice with severe combined immune deficiency

Academic Article

Abstract

  • A process unique to lymphocyte differentiation is the rearrangement of genes encoding antigen-specific receptors on B and T cells. A mouse mutant (C.B-17scid) with severe combined immune deficiency, i.e., that lacks functional B and T cells, shows no evidence of such gene rearrangements. However, rearrangements were detected in Abelson murine leukemia virus-transformed bone marrow cells and in spontaneous thymic lymphomas from C.B-17scid mice. Most of these rearrangements were abnormal: ∼80% of Igh rearrangements deleted the entire Jh region, and ∼60% of TCRβ rearrangements deleted the entire Jβ2 region. The deletions appeared to result from faulty D-to-J recombination. No such abnormal rearrangements were detected in transformed tissues from control mice. The scid mutation may adversely affect the recombinase system catalyzing the assembly of antigen receptor genes in developing B and T lymphocytes. © 1986.
  • Published In

  • Cell  Journal
  • Digital Object Identifier (doi)

    Pubmed Id

  • 25839890
  • Author List

  • Schuler W; Weiler IJ; Schuler A; Phillips RA; Rosenberg N; Mak TW; Kearney JF; Perry RP; Bosma MJ
  • Start Page

  • 963
  • End Page

  • 972
  • Volume

  • 46
  • Issue

  • 7