Alternative Pathway Complement C3 Convertase

A serine protease that is the complex of COMPLEMENT C3B and COMPLEMENT FACTOR BB. It cleaves multiple COMPLEMENT C3 into COMPLEMENT C3A (anaphylatoxin) and COMPLEMENT C3B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY.

Also Known As:

Complement C3 Convertase, Alternative Pathway; Alternative Pathway C3 Convertase; C3 Convertase (C3bBb); C3 Convertase, Alternative Pathway; CVFBb; Complement 3 Convertase, Alternative Pathway; Complement 3b-Associated Factor B-fragment Bb; Complement C3 Convertase, CVFBb; Complement 3b Associated Factor B fragment Bb

Networked: 13 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Amino Acids, Peptides, and Proteins: 1
- Proteins: 496323
  - Blood Proteins: 12679
    - Immunoproteins: 43
      - Complement System Proteins: 34043
        Complement Activating Enzymes
        Complement C3-C5 Convertases: 86
        Alternative Pathway Complement C3-C5 Convertases
        Alternative Pathway Complement C3 Convertase: 13

Experts

1.	Fremeaux-Bacchi, Veronique: 3 articles (11/2017 - 02/2009)
2.	Cailliez, Mathilde: 1 article (11/2017)
3.	Chauvet, Sophie: 1 article (11/2017)
4.	Deschenes, Georges: 1 article (11/2017)
5.	Dragon-Durey, Marie-Agnes: 1 article (11/2017)
6.	Fakhouri, Fadi: 1 article (11/2017)
7.	Fischbach, Michel: 1 article (11/2017)
8.	Karras, Alexandre: 1 article (11/2017)
9.	Le Quintrec, Moglie: 1 article (11/2017)
10.	Legendre, Christophe: 1 article (11/2017)

Related Diseases

1.	Membranoproliferative Glomerulonephritis (Membranoproliferative Glomerulonephritis, Type II) 11/01/2017 - "C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. " 10/01/1990 - "Production of IgG and IgM autoantibody to the alternative pathway C3 convertase in normal individuals and patients with membranoproliferative glomerulonephritis." 08/01/1990 - "The IgG fraction of serum from patients with membranoproliferative glomerulonephritis (MPGN) types I and II or partial lipodystrophy (PLD) was found to contain C3 nephritic factor (C3NeF) which reacts with the alternative pathway C3 convertase C3bBb and stabilizes it. " 06/15/1986 - "Nephritic factor of the alternative complement pathway (C3NeF) is an IgG autoantibody which binds to and stabilizes the C3 convertase (C3bBb) enzyme, and which has been detected mainly in sera from patients with membranoproliferative glomerulonephritis (MPGN) and partial lipodystrophy. "
2.	Glomerulonephritis 11/01/2017 - "C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. " 10/01/1990 - "Anti-idiotypic antibodies to autoantibody against the alternative pathway C3 convertase (C3NeF) were isolated and purified from normal human serum as well as from serum from six patients with membrano-proliferative glomerulonephritis (MPGN). " 02/01/2009 - "However, C3 Nephritic Factor (C3NeF), an autoantibody against the alternative C3 convertase C3bBb, and mutations in regulatory proteins of the AP have also been identified in other subtypes of MPGN and even in glomerulonephritis with mesangial C3 deposits. "
3.	Meningococcal Infections 08/01/1987 - "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." 10/01/2008 - "Many complement deficiencies, among them acquired partial C3 deficiency due to stabilizing autoantibodies against the alternative pathway C3 convertase (C3 nephritic factors, C3 NeF); increase the risk of meningococcal infection. "
4.	Lipodystrophy 08/01/1990 - "The IgG fraction of serum from patients with membranoproliferative glomerulonephritis (MPGN) types I and II or partial lipodystrophy (PLD) was found to contain C3 nephritic factor (C3NeF) which reacts with the alternative pathway C3 convertase C3bBb and stabilizes it. " 06/15/1986 - "Nephritic factor of the alternative complement pathway (C3NeF) is an IgG autoantibody which binds to and stabilizes the C3 convertase (C3bBb) enzyme, and which has been detected mainly in sera from patients with membranoproliferative glomerulonephritis (MPGN) and partial lipodystrophy. "
5.	Nephritis 06/01/1999 - "Five conditions in which the alternative pathway C3 convertase, C3b,Bb, circulates in excess as a result of factor H dysfunction are frequently accompanied by nephritis. "

Related Drugs and Biologics

1.	Autoantibodies
2.	Complement C3 Nephritic Factor
3.	Immunoglobulin G (IgG)
4.	Complement System Proteins (Complement)
5.	Complement Factor H (Factor H)
6.	Proteins (Proteins, Gene)
7.	Enzymes
8.	CD55 Antigens (Decay Accelerating Factor)
9.	Complement Factor B (Factor B)
10.	Immunoglobulin M (IgM)