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PubMed1990

Two types of C3 nephritic factor: properdin-dependent C3NeF and properdin-independent C3NeF.

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. Two types of C3NeF were detected, of which one was heat sensitive (56 degrees C for 30 min) and properdin dependent (C3NeF:P) but the other was heat stable and properdin independent (C3NeF:nP). C3NeF:P was found in sera from patients with MPGN types I and II and it displayed the properties of properdin and IgG. C3NeF:P was observed in patients with reduced serum concentrations of C3 and terminal complement components (TCC), and the generation of SC5b-9 complex was increased in mixtures with normal human serum. C3NeF:nP was found in sera from patients with MPGN type II and PLD, whose sera revealed a selective decrease in C3 concentrations.

Autoantibodies↗
PubMed1988

Production of C3 nephritic factor by cultured lymphocytes derived from a patient with partial lipodystrophy.

C3 nephritic factor (C3 NeF) has been found mainly in the sera of patients with membranoproliferative glomerulonephritis (MPGN) and partial lipodystrophy (PLD). We examined whether peripheral blood mononuclear cells (PBMC) from a patient with PLD could produce C3 NeF. We investigated the in vitro immunoglobulin synthesis of PBMC with mitogen. We further studied the C3bBb stabilizing activity and undertook agglutination assays of the IgG obtained from the culture supernatants. The patient IgG was able to agglutinate only EAC4b3bBb cells and none of the other intermediate cells. We this demonstrated that C3 NeF could be produced in vitro by PBMC derived from a patient with PLD.

Child↗
PubMed1981

Test for C3 nephritic factor activity by immunofixation electrophoresis.

This report describes a relatively simple test for C3 nephritic factor (C3NeF) activity utilizing immunofixation electrophoresis to quantitate the production of the breakdown product C3c. Tests performed on plasmas from 22 control patients and 93 renal patients have biopsy diagnoses other than basement membrane dense deposit disease (BMDDD) yielded negative results for C3NeF activity. Tests performed on plasmas from three patients with BMDDD were positive for C3NeF activity, yielding values significantly higher than those for the control group and the patients with other renal diseases. The test can be performed on specimens collected in EDTA to prevent in vitro C3 degradation during storage or transport at ambient temperature.

Basement Membrane↗
PubMed1990

Autoantibody to complement neoantigens in membranoproliferative glomerulonephritis.

With the exception of C3 nephritic factor, autoantibody formation has not been commonly associated with membranoproliferative nephritis (MPGN). We measured autoantibodies (nephritic factors) to the C3 convertases C3bBb (NFa) and C3bBbP (NFt), which result in fast and slow C3 activation, respectively, and to a neoantigen on C1q fixed to a solid phase (spC1q) in sera from 29 patients with MPGN type I, 26 with type II, and 28 with type III. Autoantibody formation was common in all MPGN types. An autoantibody to a C3 convertase neoantigen was identified in more than 75% of the hypocomplementemic MPGN sera tested. Anti-C3bBb (NFa) was present in 81% of patients with MPGN type II but was rarely found in either type I or type III. Anti-C3bBbP (NFt) was common in both MPGN I and III. Anti-spC1q was present in 74% of patients with type I and in 38% and 48% of types II and III MPGN, respectively. Patients with MPGN types I, II, and III had one and two serum autoantibodies detected significantly more frequently than did a group of healthy subjects. The presence of any one autoantibody was not specifically associated with the presence of any other autoantibody. The results indicate that multiple autoantibody formation is common in all MPGN types. MPGN II, and possibly MPGN I, tend to form more specific autoantibodies.

Antigen-Antibody Complex↗
PubMed1994

Hypocomplementaemia of poststreptococcal acute glomerulonephritis is associated with C3 nephritic factor (C3NeF) IgG autoantibody activity.

We have observed that decreased plasma levels of C3 in the serum of three children with poststreptococcal acute glomerulonephritis (PSAGN) at the time of presentation were associated with the presence of C3NeF activity in purified serum IgG from the patients. C3NeF activity was determined using a sensitive assay measuring the ability of patients' IgG to stabilize a preformed cell-bound alternative pathway convertase complex. C3NeF activity of patients' IgG decreased within weeks as plasma levels of C3 progressively returned to normal values. C3NeF activity became undetectable within 1-4 months following normalization of plasma C3 levels. Our observations suggest that early alternative pathway-dependent hypocomplementaemia, a cardinal feature of PSAGN, is mediated by the transient expression of C3NeF autoantibody activity by patients' IgG.

Acute Disease↗
PubMed1990

[Production of C3 nephritic factor by cultured lymphocytes derived from glomerulonephritic patients].

C3 nephritic factor (C3 NeF) has been found mainly in the sera of patients with membranoproliferative glomerulonephritis (MPGN) and partial lipodystrophy (PLD). We examined whether peripheral blood mononuclear cells (PBMC) from patients with PLD and MPGN could produce C3 NeF. We investigated the in vitro immunoglobulin synthesis of PBMC with mitogen. We further studied whether or not C3 NeF was included in the IgG of the culture supernatants by the C3bBb stabilizing activity and agglutination assay. The IgG of PLD patient was able to agglutinate only EAC4bBb cells and none of the other intermediate cells. We thus demonstrated that C3 NeF could be produced in vitro by PBMC derived from the patient with PLD. However, as regard case of C3 NeF weakly positive and C3 NeF negative patients, C3 NeF couldn't be produced in vitro by PBMC derived from the patients.

Adolescent↗
PubMed1992

Significance of C3 nephritic factor (C3NeF) in non-hypocomplementaemic serum with membranoproliferative glomerulonephritis (MPGN).

C3NeF is an autoantibody of C3 convertase (C3bBb) and is often detected in the serum of hypocomplementaemic MPGN patients. Serum samples from 104 non-hypocomplementaemic MPGN patients (C3NeF) were studied. C3NeF, which cannot activate the alternative pathway, was found in the sera of 6 patients. We examined the C3NeF in purified IgG from five of the non-hypocomplementaemic serum samples (non-hypo C3NeF) and four hypocomplementaemic serum samples (hypocomplementaemic C3NeF) to determine why C3NeF does not induce C3 splitting and hypocomplementaemia. Purified IgG from non-hypo C3NeF stabilized EAC4b3bBb cells in a manner similar to IgG from hypocomplementaemic C3NeF in EDTA gelatin veronal buffer. However, the non-hypo C3NeF IgG did not stabilize C3 convertase (EAC4b3bBb cells) in the presence of control proteins (factors H and I), whereas the hypocomplementaemic C3NeF IgG did. The C3NeF in the hypocomplementaemic serum displayed two characteristics: (i) inhibition of intrinsic decay of Ce convertase (C3bBb); and (ii) inhibition of extrinsic decay by factors H and I. Although the C3NeF in the non-hypocomplementaemic sera did inhibit the intrinsic decay in a manner similar to the hypocomplementaemic C3NeF IgG, it did not inhibit the extrinsic decay. Due to the different characteristics of hypocomplementaemic C3NeF and non-hypo C3NeF in the serum samples, the non-hypo C3NeF did not activate C3. Therefore, we conclude that C3NeF exhibits a heterogeneity which is very important in relation to the pathogenesis of MPGN.

Complement C3 Nephritic Factor↗
PubMed1982

Nephritic factor (NeF) of alternate pathway (NeFA) and of classical pathway (NeFc).

NeFA and NeFc were studied in various cases (115 cases). 5 cases were followed up for a long time. NeFA assay was done by the "microtest plate method". We detected the NeF activity for the first time in the cases of Sjögren syndrome (SjS) and SjS+SLE+Hashimoto's disease (Hashimoto). In some cases NeF activity disappeared after therapy, and in one case NeFA and NeFc were positive at first, but then only NeFA activity became negative following the adequate therapy. As to the antibody nature of NeF, the possibility was suggested that NeFA might be anti C3b autoantibody and NeFc might be anti C4b and/or C4b2a auto-antibody.

Complement Activation↗
PubMed1987

C3 nephritic factor determination. A comparison between two methods.

C3 nephritic factor (NEF), an IgG autoantibody to the alternative pathway C3 convertase, is usually measured by crossed immunoelectrophoresis (CI) but recently a reliable haemolytic assay (HA) was described by Rother (1982). This method is more specific than CI because it is negative in sera with immune complexes, SLE and sera incubated with IgG aggregates. The haemolytic assay is sensitive enough to detect NEF antibody in serum from patients with only slightly low C3 levels and NEF negatives by CI. The haemolytic assay is easy to perform and reproducible, the interassay coefficient of variation being 10.7% compared to 64% in the CI. The intra-assay coefficient of variation in CI was 28% compared to 5.5% in the haemolytic assay. The haemolytic method enabled us to study the kinetic effects of NEF on C3b.Bb bound to sheep erythrocytes, and the lysis mediated by ShE.C3b.Bb.NEF complex. Also the C and NEF binding to sheep erythrocytes was studied.

Animals↗
PubMed1987

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.

Hemolytic complement was found to be absent in the serum of an 11-yr-old girl (R.N.) with meningococcal septicemia. C1, C4, and C2 were slightly decreased, C3 was absent, C5-C9 within the normal range. B levels immunochemically and electrophoretic mobility of B were normal. C3d was greater than 1000% of a pooled EDTA-plasma standard indicating hypercatabolism of C3. On incubation of the patient's serum with normal human serum activation of C3 occurred even in the presence of 0.04 M EDTA. The amount of C3b generated was, however, greater without any chelating agent or in Mg-EGTA. On gel filtration of the serum two protein containing peaks were found to be responsible for activation of C3: the IgG containing peak was able to activate C3 in normal human serum without chelating agents and in Mg-EGTA but not in the presence of EDTA. The IgM-containing peak activated the third component of complement even in the presence of EDTA. The factor responsible for this phenomenon was termed C3 converting factor (C3 CoF). The IgG fraction of the patients serum caused activation of C3 in Mg-EGTA. However, in the presence of EDTA no activation of C3 could be induced even if physiological concentrations of the patients IgG were added to normal human EDTA-plasma. Thus the activity of the patient's IgG did not differ from typical C3 nephritic factor. The decay of C2 in EAC42 intermediates in the presence of the patient's IgG was uninfluenced indicating that it did not carry autoantibody activity against the classical pathway convertase C4b,2a, an activity recently termed NFc.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMedDate not supplied

[Partial lipodystrophy, hypocomplementemia and glomerulonephritis].

Renal involvement is found in 20 to 50% of cases of Partial lipodystrophy (PLD). We report 8 cases of PLD of which 6 had a glomerular nephropathy and 2 had no renal disease but all had persistent hypocomplementemia and 5 had circulating nephritic factor (C3NeF). The analysis of these cases and of all the cases reported in the literature shows the specificity of the glomerular involvement. Membranoproliferative glomerulonephritis (MPGN) with dense intramembranous deposits is a constant finding in PLD with renal involvement. This variety of MPGN is well known for being associated with persistent hypocomplementemia. However, the presence of hypocomplementemia and C3NeF in patients with PLD but without nephritis raises the question of the interrelationship between alternative pathway complement activation and the development of MPGN with or without lipodystrophy. There is no valid explanation in the present state of knowledge for the association of partial lipodystrophy hypocomplementemia, and MPGN. From the answer to this problem should emerge a better understanding of the role of complement in renal disease and in particular in the unusual form of glomerular injury seen in MPGN.

Adolescent↗
PubMed1981

Immunologic abnormalities in myelofibrosis with activation of the complement system.

Eighteen patients with agnogenic myeloid metaplasia with myelofibrosis were studied for clinical and laboratory evidence of immunologic dysfunction. Clinical findings included the presence of arthritis, vasculitis, and erythema nodosum. Laboratory abnormalities included the presence of circulating immune complexes, antinuclear antibodies, positive direct Coombs tests, elevated latex fixations, and a circulating lupus type anticoagulant. Total hemolytic complement was markedly depressed in four patients. Analysis of complement (C) components C1-C9 and factor B demonstrated significant reduction of only C3 and factor B. By crossed-immunoelectrophoresis, both C3 and factor B, but not C4, were cleaved, indicating that C activation was occurring predominantly via the alternative pathway. The control proteins beta 1H and C3b inactivator were decreased in three of four patients with hypocomplementemia. These data suggest that immunologic mechanisms associated with activation of the complement system play an important role in the disease process of some patients with agnogenic myeloid metaplasia with myelofibrosis.

Aged↗
PubMed1978

The immunogloblin nature of nephritic factor (NeF).

NeF was shown to be antigenically and structurally similar to IgG by the following experiments: (1) NeF activity in serum was absorbed by and, under acid conditions, could be eluted from (a) anti-myeloma IgG antibody coupled to Sepharose and (b) protein A-Sepharose. (2) Purified NeF could bind to anit-myeloma IgG-Sepharose and could be eluted with acid, and this binding was blocked by myeloma IgG. (3) An antibody to beta2, microglobulin, showing strong cross-reactivity with normal IgG, bound NeF activity before, but not after, absorption of the antiserum with IgG. (4) Sepharose-coupled antibodies to NeF could bind activity which was recovered in the acid eluate. This binding capacity was lost after absorption of the antibody with normal and myeloma IgG. (5) Structural similarity was demonstrated by pepsin and papain digestion, which resulted in NeF activity eluting with F(ab')2 and Fab fragments from protein A-Sepharose and Sephadex G-150. (6) Autoradiography of PAGE-SDS of 125I-labelled NeF eluted from EA43bBb cells showed that NeF had a larger H chain than normal IgG, suggesting that NeF might be an abnormal IgG molecule.

Chemical Phenomena↗
PubMed1994

Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN).

One hundred patients diagnosed with hypocomplementaemic MPGN (C3 < 40%) were studied to determine the presence of C3 nephritic factor (C3NeF) and/or C4 nephritic factor (C4NeF). Of those studied, 12 were C3NeF-positive, nine were C4NeF-positive and 10 were positive for both C3NeF and C4NeF. In the 10 patients both C3NeF- and C4NeF-positive, a marked decrease in C3 and C5 levels and a decrease in levels of late components from C6 to C9 were observed. This observation was in contrast to that seen in patients who were either C3NeF- or C4NeF-positive. Patients positive for both C3NeF and C4NeF continued to exhibit hypocomplementaemia after therapy. Immunofluorescent findings revealed heavy C3 immunoglobulin deposits in the 10 patients who were both C3NeF- and C4NeF-positive, whereas no such deposits were found in those patients who were either C3NeF- or C4NeF-positive only. When those patients who were both C3NeF- and C4NeF-positive were compared with those who were either C3NeF- or C4NeF-positive, nephritic syndrome and a poor prognosis were observed more frequently. This study demonstrates a correlation between clinical outcome and hypocomplementaemic MPGN. Further investigations of MPGN as an autoimmune disease are necessary.

Adolescent↗
PubMed1996

Sifting the causes of microscopic hematuria.

Among the goals of the primary care workup are detection of serious disease and determination of whether referral--such as to a urologist or nephrologist--is indicated. Diagnosis is facilitated when hematuria is accompanied by other findings; isolated hematuria presents a more complex challenge. Measurement of serum complement levels may be helpful in narrowing the differential.

Adult↗