The effect of the X-linked immune deficiency gene (xid) upon the Y chromosome-related disease of BXSB mice.
BXSB mice develop a lupus-like disease characterized by B cell hyperplasia, hypergammaglobulinemia, autoantibodies, nephritis and coronary artery disease. To determine the subset of B cells responsible for disease in these mice, we bred a congenic BXSB.xid strain (greater than 99.2% inbred) with the xid gene that deletes a subset of splenic B cells. Because BXSB disease is associated with the Y chromosome, BXSB males and the autoimmune cross (NZB X BXSB)F1 males were studied. BXSB.xid males had profoundly reduced lymphoid hyperplasia, hypergammaglobulinemia, autoantibodies, renal disease, cardiac disease, and markedly prolonged survival. (NZB.xid/+ X BXSB)F1 males also demonstrated a marked protection associated with the xid gene. These studies suggest that the autoimmune disease of BXSB males is dependent upon the B cell subset deleted by xid.
D000818:Animals; D001323:Autoantibodies / Q000096:biosynthesis; D001327:Autoimmune Diseases / Q000235:genetics* / Q000276:immunology / Q000401:mortality; D001789:Blood Group Antigens / Q000276:immunology; D003327:Coronary Disease / Q000209:etiology; D004277:DNA, Single-Stranded / Q000276:immunology; D005260:Female; D005434:Flow Cytometry; D007136:Immunoglobulins / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000235:genetics* / Q000276:immunology; D007674:Kidney Diseases / Q000209:etiology; D008297:Male; D051379:Mice; D008808:Mice, Inbred CBA / Q000235:genetics*; D008815:Mice, Inbred Strains / Q000235:genetics*; D009929:Organ Size; D012729:Sex Chromosome Aberrations / Q000235:genetics / Q000276:immunology; D013154:Spleen / Q000033:anatomy & histology; D014960:X Chromosome; D014998:Y Chromosome
D001323:Autoantibodies; D001789:Blood Group Antigens; D004277:DNA, Single-Stranded; D007136:Immunoglobulins