[B-cell anomalies in systemic lupus erythematosus].
Systemic lupus erythematosus is a non-organ-specific autoimmune disease characterized biologically by B lymphocyte hyperactivity and the production of autoantibodies directed against various cellular components, in particular nuclear antigens. Different strains of mice spontaneously develop a lupus-like disease and constitute a guidelight for human SLE. Both polyclonal B cell stimulation and clonal expansion induced by self-antigens participate in B cell hyperactivity observed in human and mouse SLE. B cells are hyperactive to various stimuli, in particular those delivered by T cells through surface molecules or cytokines. The consequences are an increased production of immunoglobulins and the development of autoantibodies thought to induce the major part of tissue lesions. B cells also participate in the pathological process as antigen-presenting and cytokine-secreting cells. An intrinsic defect of B cells is suspected to be responsible for B cell anomalies as illustrated by certain spontaneous murine models of SLE (motheaten mice) and by lupus-like syndromes observed in mice rendered deficient for genes controlling the B-cell receptor (BCR) signaling pathway. Genome wide scan analysis of various lupus strains allowed to identify several loci predisposing to lupus among which certain are associated with B cell hyperactivity suggesting that the intrinsic defect is inherited.
Tron
François
F
Unité INSERM U519, Institut Fédératif de Recherches Multidisciplinaires sur les Peptides (IFR23), Faculté Mixte de Médecine et Pharmacie, CHU Charles-Nicolle, 22, boulevard Gambetta, 76183 Rouen Cedex 1. francois.tron@chu-rouen.fr
Jacquot
Serge
S
Gilbert
Danièle
D
D000818:Animals; D000974:Antibodies, Antinuclear / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D016207:Cytokines / Q000276:immunology; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D007111:Immunity, Cellular; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology*; D051379:Mice; D023421:Models, Animal; D011947:Receptors, Antigen, B-Cell / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology
D000974:Antibodies, Antinuclear; D016207:Cytokines; D011947:Receptors, Antigen, B-Cell