The lupus-related Lmb3 locus contains a disease-suppressing Coronin-1A gene mutation

Immunity. 2008 Jan;28(1):40-51. doi: 10.1016/j.immuni.2007.11.023.

Abstract

Here, we show that a lupus-suppressing locus is caused by a nonsense mutation of the filamentous actin-inhibiting Coronin-1A gene. This mutation was associated with developmental and functional alterations in T cells including reduced migration, survival, activation, and Ca2+ flux. T-dependent humoral responses were impaired, but no intrinsic B cell defects were detected. By transfer of T cells, it was shown that suppression of autoimmunity could be accounted for by the presence of the Coro1a(Lmb3) mutation in T cells. Our results demonstrate that Coronin-1A is required for the development of systemic lupus and identify actin-cytoskeleton regulatory proteins as potential targets for modulating autoimmune diseases.

Publication types

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

MeSH terms

  • Actins / metabolism
  • Adoptive Transfer
  • Animals
  • Apoptosis / physiology
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • B-Lymphocytes / immunology
  • Cell Proliferation
  • Chemotaxis, Leukocyte / immunology
  • Codon, Nonsense
  • Enzyme-Linked Immunosorbent Assay
  • Flow Cytometry
  • Genetic Predisposition to Disease*
  • Immunoglobulin G / blood
  • Immunoglobulin G / immunology
  • Immunohistochemistry
  • Lupus Erythematosus, Systemic / genetics*
  • Lupus Erythematosus, Systemic / immunology
  • Lymphocyte Activation / immunology
  • Mice
  • Microfilament Proteins / genetics*
  • Polymerase Chain Reaction
  • T-Lymphocytes / immunology*
  • Transfection

Substances

  • Actins
  • Autoantibodies
  • Codon, Nonsense
  • Immunoglobulin G
  • Microfilament Proteins
  • coronin proteins