Defective development and function of Bcl10-deficient follicular, marginal zone and B1 B cells

Nat Immunol. 2003 Sep;4(9):857-65. doi: 10.1038/ni963. Epub 2003 Aug 10.


Bcl10 is an intracellular protein essential for nuclear factor (NF)-kappaB activation after lymphocyte antigen receptor stimulation. Using knockout mice, we show that absence of Bcl10 impeded conversion from transitional type 2 to mature follicular B cells and caused substantial decreases in marginal zone and B1 B cells. Bcl10-deficient B cells showed no excessive apoptosis. However, both Bcl10-deficient follicular and marginal zone B cells failed to proliferate normally, although Bcl10-deficient marginal zone B cells uniquely failed to activate NF-kappaB efficiently after stimulation with lipopolysaccharide. Bcl10-deficient marginal zone B cells did not capture antigens, and Bcl10-deficient (Bcl10-/-) mice failed to initiate humoral responses, leading to an inability to clear blood-borne bacteria. Thus, Bcl10 is essential for the development of all mature B cell subsets.

Publication types

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

MeSH terms

  • Adaptor Proteins, Signal Transducing*
  • Animals
  • Apoptosis / immunology*
  • B-Cell CLL-Lymphoma 10 Protein
  • B-Lymphocyte Subsets / immunology
  • B-Lymphocytes / cytology
  • B-Lymphocytes / immunology*
  • Bone Marrow / immunology
  • Carrier Proteins / genetics
  • Carrier Proteins / immunology*
  • Cell Division / immunology
  • Female
  • Flow Cytometry
  • Immunohistochemistry
  • In Situ Nick-End Labeling
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • NF-kappa B / immunology*
  • Pneumococcal Infections / immunology
  • Streptococcus pneumoniae


  • Adaptor Proteins, Signal Transducing
  • B-Cell CLL-Lymphoma 10 Protein
  • Bcl10 protein, mouse
  • Carrier Proteins
  • NF-kappa B