A properdin dependent nephritic factor slowly activating C3, C5, and C9 in membranoproliferative glomerulonephritis, types I and III.
The IgG fraction of serum from patients with membranoproliferative glomerulonephritis (MPGN) types I and III was found to contain a nephritic factor (NFI/III) which differed from that usually present in MPGN type II and partial lipodystrophy (NFII) in that it converts C5 and C9 as well as C3, is dependent on properdin for its activity, and requires an incubation period of several hours rather than 30 min for its demonstration. C3 conversion occurred in the absence of an intact classical pathway. This nephritic factor was found in patients with reduced serum levels of terminal components and its activity, like that of the nephritic factor in MPGN type II, correlated with the serum C3 level indicating that these nephritic factors play a large role in producing hypocomplementemia. Although potentially nephritogenic because of its ability to activate the terminal pathway, the presence of this nephritic factor did not clearly correlate with clinical course.
Clardy
C W
CW
Children's Hospital Research Foundation, Cincinnati, Ohio 45229-2899.
Forristal
J
J
Strife
C F
CF
West
C D
CD
D003167:Complement Activation; D003176:Complement C3 / Q000172:deficiency / Q000378:metabolism; D003178:Complement C3 Nephritic Factor / Q000302:isolation & purification / Q000502:physiology*; D003182:Complement C5 / Q000378:metabolism; D003186:Complement C9 / Q000378:metabolism; D003169:Complement Inactivator Proteins / Q000502:physiology*; D003170:Complement Pathway, Alternative; D015432:Glomerulonephritis, Membranoproliferative / Q000097:blood* / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000302:isolation & purification / Q000502:physiology; D011414:Properdin / Q000502:physiology*
D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003182:Complement C5; D003186:Complement C9; D003169:Complement Inactivator Proteins; D007074:Immunoglobulin G; D011414:Properdin