Altered B cell receptor signaling in human systemic lupus erythematosus

Autoimmun Rev. 2009 Jan;8(3):209-13. doi: 10.1016/j.autrev.2008.07.047. Epub 2008 Aug 22.

Abstract

Regulation of B cell receptor signaling is essential for the development of specific immunity while retaining tolerance to self. Systemic lupus erythematosus (SLE) is characterized by a loss of B cell tolerance and the production of anti-self antibodies. Accompanying this break down in tolerance are alterations in B cell receptor signal transduction including elevated induced calcium responses and increased protein phosphorylation. Specific pathways that negatively regulate B cell signaling have been shown to be impaired in some SLE patients. These patients have reduced levels of the kinase Lyn in lipid raft microdomains and this reduction is inversely correlated with increased CD45 in lipid rafts. Function and expression of the inhibitory immunoglobulin receptor FcgammaRIIB is also reduced in Lupus IgM- CD27+ memory cells. Because the relative contribution of different memory and transitional B cell subsets can be abnormal in SLE patients, we believe studies targeted to well defined B cell subsets will be necessary to further our understanding of signaling abnormalities in SLE. Intracellular flow cytometric analysis of signaling is a useful approach to accomplish this goal.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Autoantibodies / immunology*
  • B-Lymphocyte Subsets / enzymology*
  • B-Lymphocyte Subsets / immunology
  • B-Lymphocyte Subsets / pathology
  • B-Lymphocytes / enzymology*
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology
  • Cell Separation / methods
  • Flow Cytometry / methods
  • Humans
  • Immunologic Memory
  • Lipid Metabolism / immunology
  • Lupus Erythematosus, Systemic / immunology
  • Lupus Erythematosus, Systemic / metabolism
  • Lupus Erythematosus, Systemic / pathology
  • Phosphorylation
  • Receptors, Antigen, B-Cell / immunology
  • Receptors, Antigen, B-Cell / metabolism*
  • Receptors, IgG / genetics
  • Receptors, IgG / immunology
  • Receptors, IgG / metabolism*
  • Self Tolerance
  • Signal Transduction
  • src-Family Kinases / immunology
  • src-Family Kinases / metabolism

Substances

  • Autoantibodies
  • FCGR2B protein, human
  • Receptors, Antigen, B-Cell
  • Receptors, IgG
  • lyn protein-tyrosine kinase
  • src-Family Kinases