title: Glomerular deposition of complement-control proteins in acute and chronic glomerulonephritis.
abstract: Acute poststreptococcal glomerulonephritis (AGN) differed from membranoproliferative glomerulonephritis (MPGN) and lupus nephritis (SLE) in that two of the proteins that control the C3b-dependent convertase, beta 1H and the C3bC4b-inactivator cofactor (C3bC4bICo), were frequently absent from the glomerular deposits. In addition, factor B was distributed with C3 in the capillary walls in hypocomplementemic AGN patients. From this, it can be assumed that C3bBb is in the deposits, uninhibited by control proteins as would be predicted for alternative pathway activation. Factor B could not be found in normocomplementemic AGN, was rarely present in MPGN, but was usually present in SLE, most often in the mesangium. In MPGN and SLE, the control proteins were nearly always present in the glomeruli in a distribution like that of C3; IN MPGN they were particularly abundant. Complement profiles indicated an occasional transient reduction in serum C4 level early in AGN. Thus, although there is occasional evidence of early classical activation in AGN, more characteristic is a long period of alternative activation. Serum levels of control proteins did not deviate greatly from normal except for reduced serum beta 1H levels in MPGN type I.
mesh_terms: D001707:Biopsy, Needle; D003167:Complement Activation; D050577:Complement C3-C5 Convertases / Q000032:analysis; D003180:Complement C3b Inactivator Proteins / Q000032:analysis; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000276:immunology*; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology; D007158:Immunologic Techniques; D007678:Kidney Glomerulus / Q000276:immunology* / Q000473:pathology
chemical_list: D003180:Complement C3b Inactivator Proteins; D007136:Immunoglobulins; D003165:Complement System Proteins; D050577:Complement C3-C5 Convertases