Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders
C3 glomerulopathy is a glomerular kidney disease caused by abnormal activation of the alternative complement pathway. The dominating histomorphological finding is C3 deposition. According to electron microscopy of the localization of these deposits, two subtypes can be distinguished dense deposit disease and C3 glomerulonephritis.
| Parameter | Interpretation |
|---|---|
| ANA, ANCA, Anti-GBM | Autoantibodies |
| HIV, Hepatitis B and C | Infectiousness |
| HDL-C, LDL-C, Chol, TG | Lipids |
| Serum albumin und total protein | nutrition/protein loss |
| IgG (subtypes), IgM, IgA, IgE | Immunoglobulin abnormalities |
| Albuminuria/g Crea | Urine-protein-to-creatinine ratio (UPr/Cr) |
| Parameter | Interpretation |
|---|---|
| C3, C4, C3d | Complement activation |
| CH50, APH50 | total hemolytic complement activity, classical and alternative |
| sMAC/CD5b9 | soluble membrane attack complex |
| C3NeF | C3-convertase of the alternative pathway-(C3bBb)-autoantibodies |
| Factors I, B, D, H and CFHR1-5 | modulators of the complement cascade |
| autoantibodies factor B, H, CFHR | autoantibodies of modulators |
In rare cases autoantibodies against C3-convertase of the classical pathway-(C4bC2b)is detectable. Also rare are atoantibodies against C3 and Non-c3NeF-autoantibodies against different components of the C3-convertase. Those antibodies, because so rare are difficult to interpret nephrologically.
Klinisches Register für C3 Glomerulopathie und Immunkomplex-vermittelte MPGN
| 1. |
Brai M et al. (1988) Combined homozygous factor H and heterozygous C2 deficiency in an Italian family. 
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| 2. |
Barbour TD et al. (2013) Dense deposit disease and C3 glomerulopathy.
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| 3. |
Chen Q et al. (2014) Complement factor H-related hybrid protein deregulates complement in dense deposit disease.
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| 4. |
Redahan L et al. (2014) Familial MPGN - a case series: a clinical description of familial membranoproliferative glomerulonephritis amongst three Irish families.
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Xiao X et al. (2014) C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis.
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Appel GB et al. (2005) Membranoproliferative glomerulonephritis type II (dense deposit disease): an update.
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None (2002) Complement in glomerulonephritis.
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| 8. |
Fijen CA et al. (1996) Heterozygous and homozygous factor H deficiency states in a Dutch family.
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Wyatt RJ et al. (1982) Partial H (beta 1H) deficiency and glomerulonephritis in two families.
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Nielsen HE et al. (1989) Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease.
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| 11. |
Servais A et al. (2007) Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome.
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| 12. |
Licht C et al. (2006) Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II).
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| 13. |
Abrera-Abeleda MA et al. (2006) Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease).
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| 14. |
Dragon-Durey MA et al. (2004) Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases.
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| 15. |
Hegasy GA et al. (2002) The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion.
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| 16. |
Pickering MC et al. (2002) Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H.
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| 17. |
None (2000) Factor H and the pathogenesis of renal diseases.
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| 18. |
Sánchez-Corral P et al. (2000) Molecular basis for factor H and FHL-1 deficiency in an Italian family.
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| 19. |
Ault BH et al. (1997) Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism.
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| 20. |
Høgåsen K et al. (1995) Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency.
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| 21. |
Vogt BA et al. (1995) Inherited factor H deficiency and collagen type III glomerulopathy.
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| 22. |
Levy M et al. (1986) H deficiency in two brothers with atypical dense intramembranous deposit disease.
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| 23. |
Orphanet article Orphanet ID 329918
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