Phenotypes for Tg(Tnfsf13b)1Fma MGI:3841104 MGI Mouse

increased B cell number ( J:113556 )

• splenic B cell numbers are about twice that of controls

increased transitional stage B cell number ( J:113556 )

• numbers of T1 B cells in the spleen are increased almost 3-fold while T2 numbers are almost about doubled

abnormal spleen marginal zone morphology ( J:113556 )

• the size of the marginal zone is increased

increased marginal zone B cell number ( J:113556 )

• the number of splenic marginal zone B cells is increased by about 3-fold

abnormal B cell activation ( J:113556 )

• B cells have a vigorous response to T cell independent antigens producing much higher levels of IgG1, IgG2a, IgG2b, IgG3, and IgA class immunoglobulins than controls

abnormal marginal zone B cell physiology ( J:113556 )

• marginal zone B cells (MZB) mediate autoimmune disease in these transgenic mice as demonstrated by auto-antibody production when MZB are transferred into transgenic mice on a B-cell deficient background

• MZB from transgenic mice have higher integrin adhesion activity

increased anti-double stranded DNA antibody level ( J:113556 )

• anti-dsDNA antibodies of the IgM class, IgA class, and all IgG subclasses are found in circulation

• titers of these auto-antibodies increase with

• splenectomy at three weeks of age significantly reduces IgG auto-antibodies and partially reduces auto-antibodies in the other subclasses

glomerulonephritis ( J:113556 )

• kidneys of 10-month old mice have necrotic lesions

increased urine protein level ( J:113556 )

• all 8-month old mice have proteinuria in their urine with most having 100-300 mg/dl