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.