Accelerated decay of the cell bound C4b2a complex by serum of patients with membranoproliferative glomerulonephritis and acute poststreptococcal glomerulonephritis.
Serum from patients with membranoproliferative glomerulonephritis (MPGN) and acute poststreptococcal glomerulonephritis (APSGN) accelerated the decay of the cell bound C4b2a (C42) and C4b hemolytic activity relative to pooled normal human serum (pNHS) after 5 min incubation at 30 degrees C in EDTA-GVB. The accelerated decay of the C42 hemolytic activity was heat stable (56 degrees C 30 min) and was inhibited by monoclonal antibody against human C4 binding protein (MoAb:C4BP) or C4 binding protein (C4BP) depleted serum. C4 nephritic factor (C4NeF) was employed to stabilize the labile classical pathway C3 convertase C42 complex. Serum from patients with MPGN and APSGN reduced the C4NeF stabilizing activity. Sera from 32 of 46 patients with MPGN and all of 7 patients with APSGN reduced the C42 hemolytic activity relative to 50 normal human serum (NHS) after 5 min incubation at 30 degrees C in EDTA-GVB, and there was no relationship with the serum concentration of C4BP. In vivo, accelerated decay of C42 convertase might interfere with the clearing and processing mechanism of circulating immune complexes (IC) by reducing deposition of C3b on the IC lattice.
Tanuma
Y
Y
Second Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan.
Ohi
H
H
Hatano
M
M
D000208:Acute Disease; D001323:Autoantibodies / Q000378:metabolism*; D002352:Carrier Proteins / Q000097:blood; D003165:Complement System Proteins / Q000378:metabolism*; D005921:Glomerulonephritis / Q000097:blood* / Q000382:microbiology; D015432:Glomerulonephritis, Membranoproliferative / Q000097:blood*; D006461:Hemolysis; D006801:Humans; D016167:Integrin alphaXbeta2; D008180:Lupus Erythematosus, Systemic / Q000097:blood; D013290:Streptococcal Infections
D001323:Autoantibodies; D002352:Carrier Proteins; D016167:Integrin alphaXbeta2; C058872:complement 4 nephritic factor; D003165:Complement System Proteins