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The alternative pathway C3 convertase and glomerular deposits.
1Division of Nephrology, Children's Hospital Medical Center, Cincinnati, OH 45229-3039, USA.
Pediatric Nephrology (Berlin, Germany)
|July 21, 1999
Summary
Excess complement C3 convertase, C3b,Bb, due to factor H dysfunction causes nephritis. This occurs in Marder disease, factor H deficiency, and specific types of membranoproliferative glomerulonephritis (MPGN).
Area of Science:
- Immunology
- Nephrology
- Complement System Biology
Background:
- Factor H dysfunction leads to excess alternative pathway C3 convertase (C3b,Bb), frequently causing nephritis.
- This condition is observed in Marder disease (factor H binding site defect), homozygous factor H deficiency, factor H inhibitors, and specific MPGN types.
Purpose of the Study:
- To investigate the relationship between excess complement convertase and glomerular deposits in various nephritic conditions.
- To analyze the correlation between complement levels and the presence/type of glomerular deposits in MPGN types II and III, factor H deficiency, and Marder disease.
Main Methods:
- Observational study analyzing clinical data and biopsy findings.
- Correlation of complement levels (hypocomplementemic vs. normocomplementemic) with the presence and location of glomerular deposits.
Main Results:
- In MPGN type II, subepithelial paramesangial deposits correlate with hypocomplementemia.
- MPGN type III shows paramesangial deposits with hypocomplementemia and subendothelial deposits only during hypocomplementemia.
- Paramesangial deposits are characteristic of factor H deficiency and Marder disease.
Conclusions:
- Excessive C3 convertase is linked to nephritis and specific glomerular deposition patterns.
- Variations in nephritis incidence, severity, and morphology may depend on convertase characteristics and C3 products.