Complement C3 Nephritic Factor

An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.

Also Known As:

C 3 Nephritic Factor; C3 Nephritic Factor; Complement 3 Nephritic Factor; Autoantibody, C3NeF IgG; IgG Autoantibody, C3NeF; C3 NeF; C3 NeF IgG Autoantibodies; C3 NeF IgG Autoantibody; C3NeF IgG Autoantibodies; C3NeF IgG Autoantibody

Networked: 82 relevant articles (2 outcomes, 3 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Frémeaux-Bacchi, Véronique: 6 articles (10/2022 - 09/2011)
2.	Servais, Aude: 4 articles (10/2022 - 09/2011)
3.	Le Quintrec, Moglie: 3 articles (01/2018 - 08/2012)
4.	Baudouin, Véronique: 2 articles (10/2022 - 01/2018)
5.	Chauvet, Sophie: 2 articles (10/2022 - 06/2015)
6.	Daugas, Eric: 2 articles (10/2022 - 01/2018)
7.	Delmas, Yahsou: 2 articles (10/2022 - 01/2018)
8.	Karras, Alexandre: 2 articles (10/2022 - 08/2012)
9.	Dragon-Durey, Marie-Agnès: 2 articles (06/2015 - 08/2012)
10.	Moulin, Bruno: 2 articles (06/2015 - 08/2012)

Related Diseases

1.	Membranoproliferative Glomerulonephritis (Membranoproliferative Glomerulonephritis, Type II) 02/01/1990 - "She also had hypocomplementemia, mesangiocapillary glomerulonephritis type II, and a serum assay with positive results for C3 nephritic factor. " 03/01/1996 - "The aim of this multicentre study was to analyse the long-term outcome of idiopathic membranoproliferative glomerulonephritis (MPGN) according to histological type and to the presence of C3 nephritic factor. " 01/01/2017 - "C3 nephritic factor was detected in 2 patients from the C3-GP (dense deposit disease) group. " 02/01/2015 - "C3 nephritic factor (C3NeF) has been described in association with membranoproliferative glomerulonephritis and is involved in 80 % of cases of dense deposit disease. " 08/01/2012 - "A total of 53 patients presented with a low C3 level, and 65 were positive for C3 nephritic factor that was significantly more frequently detected in patients with dense deposit disease than in other histological types. "
2.	Proteinuria 01/01/1989 - "Plasmapheresis and chemotherapy resulted in a decrease in paraprotein concentration up to 90%, a decrease in C3 NeF-like activity to negligible, normal serum complement levels and a marked improvement in both renal function and proteinuria. " 06/01/1989 - "We have studied a girl seven and a half years old with a renal disease manifested by proteinuria and nephrotic syndrome combined with initial macroscopic hematuria normal blood pressure and persistent depression of the complement C3 levels with the complement activating gamma globulin (C3 Nef) present in serum. "
3.	Lipodystrophy 04/01/1995 - "Lupus, C3 nephritic factor and partial lipodystrophy." 09/01/1988 - "Production of C3 nephritic factor by cultured lymphocytes derived from a patient with partial lipodystrophy." 12/01/1997 - "Membranoproliferative glomerulonephritis was the predominant diagnosis and two patients had partial lipodystrophy reflecting the well-known association between these diseases and C3 NeF. " 04/01/1990 - "The occurrence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonephritis, whether singly or in any combination, is usually sporadic. " 04/01/1990 - "Familial incidence of C3 nephritic factor, partial lipodystrophy and membranoproliferative glomerulonephritis."
4.	Systemic Lupus Erythematosus (Libman-Sacks Disease) 06/01/1985 - "C3 nephritic factor (C3NeF) was used to assess the formation of the fluid-phase amplification convertase, C3b,Bb, in 37 serum specimens from 24 patients with systemic lupus erythematosus (SLE). " 01/01/1995 - "Hypocomplementaemia, C3 nephritic factor and type III mesangiocapillary glomerulonephritis progressing to systemic lupus erythematosus." 09/01/1980 - "This study involves a patient who had classic HG with C3 at the BMZ, and who had a circulating factor with activity via the alternative complement pathway similar to the previously described C3 nephritic factor found in patients with partial lipodystrophy, systemic lupus erythematosus, and membranoproliferative glomerulonephritis. " 03/27/1990 - "No C4NeF was detected in two C3 nephritic factor-positive patients with MPGN type II and six with active systemic lupus erythematosus. "
5.	Hemolysis 10/01/1977 - "In the present study we have shown that when guinea pig erythrocytes were incorporated in agar and normal human serum was allowed to diffuse toward C3 nephritic factor-containing serum, a line of hemolysis appeared that coincided with the stainable line. " 10/01/1977 - "The line of hemolysis only formed in the presence of C3, factor B and Mg++, as does the properdin or C3 nephritic factor-induced stainable line. "

Related Drugs and Biologics

1.	Complement System Proteins (Complement)
2.	Paraproteins
3.	Agar
4.	Autoantibodies
5.	Complement C3 Nephritic Factor
6.	Complement Factor H (Factor H)
7.	Alternative Pathway Complement C3 Convertase
8.	Immunoglobulin G (IgG)
9.	Complement C3-C5 Convertases (C3 Convertase)
10.	Dichlorodiphenyldichloroethane (DDD)

Related Therapies and Procedures

1.	Plasmapheresis
2.	Drug Therapy (Chemotherapy)
3.	Kidney Transplantation
4.	Therapeutics
5.	Peritoneal Dialysis