Persistently circulating C3 nephritic factor (C3 NeF)-stabilized alternative pathway C3 convertase (C3 CoF) in serum of an 11-year-old girl with meningococcal septicemia--simultaneous occurrence with free C3 NeF.
Hemolytic complement was found to be absent in the serum of an 11-yr-old girl (R.N.) with meningococcal septicemia. C1, C4, and C2 were slightly decreased, C3 was absent, C5-C9 within the normal range. B levels immunochemically and electrophoretic mobility of B were normal. C3d was greater than 1000% of a pooled EDTA-plasma standard indicating hypercatabolism of C3. On incubation of the patient's serum with normal human serum activation of C3 occurred even in the presence of 0.04 M EDTA. The amount of C3b generated was, however, greater without any chelating agent or in Mg-EGTA. On gel filtration of the serum two protein containing peaks were found to be responsible for activation of C3: the IgG containing peak was able to activate C3 in normal human serum without chelating agents and in Mg-EGTA but not in the presence of EDTA. The IgM-containing peak activated the third component of complement even in the presence of EDTA. The factor responsible for this phenomenon was termed C3 converting factor (C3 CoF). The IgG fraction of the patients serum caused activation of C3 in Mg-EGTA. However, in the presence of EDTA no activation of C3 could be induced even if physiological concentrations of the patients IgG were added to normal human EDTA-plasma. Thus the activity of the patient's IgG did not differ from typical C3 nephritic factor. The decay of C2 in EAC42 intermediates in the presence of the patient's IgG was uninfluenced indicating that it did not carry autoantibody activity against the classical pathway convertase C4b,2a, an activity recently termed NFc.(ABSTRACT TRUNCATED AT 250 WORDS)
Wahn
V
V
Universitäts-Kinderklinik, Düsseldorf, FRG.
Müller
W
W
Rieger
C
C
Rother
U
U
D002648:Child; D003166:Complement Activating Enzymes / Q000097:blood*; D003178:Complement C3 Nephritic Factor / Q000097:blood*; D050577:Complement C3-C5 Convertases / Q000097:blood*; D003169:Complement Inactivator Proteins / Q000097:blood*; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000378:metabolism; D005260:Female; D006461:Hemolysis; D006801:Humans; D008589:Meningococcal Infections / Q000097:blood / Q000276:immunology*; D018805:Sepsis / Q000097:blood / Q000276:immunology*
D003178:Complement C3 Nephritic Factor; D003169:Complement Inactivator Proteins; D003165:Complement System Proteins; D003166:Complement Activating Enzymes; D050577:Complement C3-C5 Convertases