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. 2011 Feb;178(2):462–471. doi: 10.1016/j.ajpath.2010.09.044

Table 1.

B-Cell–Associated Humoral Autoimmunity Models and Proposed Mechanisms of Disease

Proposed mechanism Mouse genotype Autoimmune phenotype References
Promote survival of autoreactive GC B cells through the extrinsic apoptotic pathway lpr and/or gld Lymphoproliferation, autoimmunoglobulin production, multiorgan lymphocyte infiltration and disease, glomerulonephritis 72–74
CD19-conditional Fas−/− Lymphoproliferation, abnormal GC activity, lymphadenopathy, autoimmunoglobulin-mediated destruction of kidney, liver, and lungs 75
Promote survival of autoreactive GC B cells through the intrinsic apoptotic pathway Bcl-2 Tg Abnormal GC activity, enhanced cell survival, spontaneous production of autoimmunoglobulins, lupus-like glomerulonephritis. 76–78
bim−/− Abnormal GC B cell survival and selection, expanded plasma cell compartment, autoimmunoglobulin production, fatal glomerulonephritis 79,80
Abnormal B-cell signaling and proliferation CD22−/− Anti-dsDNA, cardiolipin, myeloperoxidase IgG 81
PD-1−/− IgG3-mediated arthritis, glomerulonephritis, exacerbation of lpr phenotype 82
FcγRIIB−/− Antinuclear immunoglobulins, fatal autoimmune glomerulonephritis, multiorgan inflammation 60
CD19-conditional Ptpn6 (Shp1 deficient) Unregulated B-cell proliferation, altered immunoglobulin responses, anti-DNA immunoglobulins, glomerulonephritis 61
PKCδ−/− Spontaneous GCs, unregulated proliferation, autoimmunoglobulin-mediated glomerulonephritis, multiorgan lymphocyte infiltration 62
CD19 Tg Breakdown of peripheral tolerance and production of anti-HEL autoimmunoglobulins 83
BAFF Tg Abnormal GCs, high levels of rheumatoid factors, circulating immune complexes, anti-DNA autoimmunoglobulins, deposition in the kidneys 84
Lyn−/− Impaired B-cell signaling and proliferation, increased serum immunoglobulin, antinuclear IgG, glomerulonephritis 85
AID−/− Autoimmune gastritis, exacerbation of BL/6/lpr phenotype, anti-dsDNA IgM, abnormal GC function, variable disease outcomes 50,51,57,59,96