Evidence for glomerular modulation of complement activation.
Using indirect immunofluorescence, three regulatory proteins of the complement system, C1-inhibitor (C1-INH), C3b inactivator (C3bINA) and beta 1H globulin have been detected in the glomeruli of patients with glomerulonephritis associated with complement activation. C1-INH and beta 1H were found frequently but C3bINA was rarely detected (only 14 biopsies). beta 1H globulin and C3b inactivator which modulate C3b activity were never found in the absence of C3, but C1-INH was sometimes found in the absence of C1s. The patterns for staining for C1s and C1-INH, C3 and beta 1H were almost identical suggesting that the regulatory proteins are binding to the proteins they regulate, as has been demonstrated in vitro. Thus, in tissues undergoing complement-mediated tissue damage, the extent of complement activation is controlled by the normal regulatory mechanisms.
D003167:Complement Activation / Q000276:immunology*; D003174:Complement C1 Inactivator Proteins / Q000032:analysis; D003180:Complement C3b Inactivator Proteins / Q000032:analysis; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D007678:Kidney Glomerulus / Q000276:immunology*; D011414:Properdin / Q000032:analysis
D003174:Complement C1 Inactivator Proteins; D003180:Complement C3b Inactivator Proteins; D011414:Properdin