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PubMed1990

The spleen is not a major synthetic site of alternative pathway components.

The serum concentrations of the complement proteins C1q, C1s, C4, C3, factor D, factor B, properdin, C1 inactivator, factor I and factor H were determined in 7 patients before and after splenectomy. The concentrations of C1q, factor D and properdin were moderately decreased 1-2 days after surgery, while the other components were virtually unchanged. During the course of 4 weeks the values either returned to normal, or were slightly increased. In general, the findings could be ascribed to the inflammatory plasma protein response and were similar in cholecystectomized patients. The results provided no evidence of significant complement protein synthesis in the human spleen.

Adult↗
PubMed1975

Complete absence of the third component of complement in man.

A 4-yr-old female patient who has recurrent infections with encapsulated bacteria and gramnegative organisms was found to have a complete absence of total hemolytic complement and C3. Total hemolytic complement was reconstituted by the addition of functionally pure C3. With the exception of a moderately reduced homolytic C4, all other C components, measured homolytically and by radial immunodiffusion, were present in normal amounts. By Ouchterlong analysis, the patient's serum contained C3b inactivator and properdin but no antigenic C3. Activation of the alternate pathway was examined by purified cobra venom factor (CVF) and inulin. Neither of these substances led to activation of properdin factor B to B. On addition of partially purified Cordis C3, in four out of four instances and with different preparations of Cordis C3, activation of factor B to B occurred in the inulin-serum-C3 mixture. In contrast, activation of factor B to B occurred only once out of four times with CVF-serum-C3 mixtures. Immune adherence was found to be normal in the patient's serum and could be removed by anti-C4 antiserum of hydrazine treatment. A marked opsonic defect was present against Escherichia coli. Serum bactericidal activity against a rough strain of E. coli was also defective. The ability to mobilize an infalmmatory response was examined by Rebuck skin window technique. A delay in neutrophil migration occurred until the 6th h. In vitro lymphocyte transformation and serum immunoglobulins were normal. The proportion of peripheral blood T cells forming spontaneous sheep erythrocyte rosettes and the percentage of B cells forming EAC rosettes by the C3 receptor were normal. The significance of the absence of C3 in our patient is emphasized by the increased number of infections with encapsulated bacteria and the decreased functional biological activities of the C system, important in host defense mechanism(s).

Blood Bactericidal Activity↗
PubMed1981

Complement in pneumococcal infections with varying degrees of severity.

Complement component levels (Clq, Cls, C4, C3, factor B and properdin) and C1 subcomponent complexes (C1r-C1s, C1-r-C1-, C1-r-C1-s-C1 inactivator, 1A) were studied in 16 adults with pneumococcal infections varying severity. Patients with fulminant disease and signs of septic shock showed pronounced hypocomplementemia. In patients with pneumococcal pneumonia or meningitis elevated levels of C1-r-C1-s-C1- IA complexes indicated activation of C1, despite normal levels of C1q, C1s, C4 and C3. Moderately decreased properdin values suggested involvement of the alternative pathway. In adults with pneumococcal otitis no changes in the complement profile was found. In contrast, pronounced aberrations of the C1 subcomponents were earlier demonstrated in children with otitis.

Adolescent↗
PubMed1990

The complements and immunoglobulins in different media of healthy pregnant women and in pregnant women with increased blood pressure.

We have determined the levels of complement C3, C4 and immunoglobulin G, M, A in mothers' and cord blood serum. Parallelly properdin factor B and immunoglobulin G tests were done in urine samples. All estimations were performed on Immunochemistry Analyzer "Beckman". The investigations were made on 30 healthy pregnant women and 30 with arterial hypertension at the end of third trimester. In the mothers' serum C3 was not significantly changed. In the cord blood of healthy pregnant women it was 0.69 g/L. (SD 0.12) and in those with hypertension 0.38 g/L. (SD 0.15), which means significantly decreased. Complements C4 was not significantly increased in mothers' and cord blood serum. Properdin factor B was significantly increased in mothers' and cord blood serum (healthy pregnant women in serum 0.38 g/L., SD 0.07; with hypertension 0.48 g/L., SD 0.15; while in the cord blood serum of healthy women it was 0.14 g/L., SD 0.06; and hypertensive it was 0.22 g/L., SD 0.10). The same parameter was significantly decreased in the urine of healthy subjects 3.94 mg/L., SD 1.91; and in the hypertensive too, 2.42 mg/L., SD 0.90. The IgG level was significantly increased in the urine of healthy pregnant women 4.42 mg/L., SD 2.24; with hypertension 6.64 mg/L., SD 3.61. IgM was not significantly changed in mothers' and cord blood serum. IgA was significantly increased in the cord blood serum of healthy mothers', 0.02 g/L., SD 0.01, with hypertension 0.12 g/L., SD 0.05.

Complement C3↗
PubMed1978

Acute nephritis and pulmonary alveolitis following pneumococcal pneumonia.

Acute glomerulonephritis developed in a man with pneumococcal pneumonia. Serum complement studies revealed decreased levels of C4, properdin, and C3. Renal immunofluorescence studies demonstrated pneumococcal antigen, C1q, C4, C3 proactivator, properdin, C3, IgG, and IgM. Circulating cryoglobulin contained pneumococcal antigen and antibody, C3, and immunoglobulins. Serial pneumococcal antigen and antibody levels did not display patterns that were characteristic of classical immune elimination, but the patterns may have been influenced by the reentry of antigen. A diffuse, pulmonary alveolitis also developed in the patient. Lung immunofluorescence studies revealed pneumococcal antigen, IgG, and C3 in alveolar walls and capillary basement membranes. The glomerulonephritis and alveolitis resolved after a prolonged course. These findings provide presumptive evidence for pneumococcal, immune complex glomerulonephritis with complement activation via both classical and alternative pathways and suggest an immunologic pathogenesis for the pulmonary alveolitis.

Adult↗
PubMed1979

A comparison of immunologic profiles and their influence on bacteremia in surgical patients with a high risk of infection.

Prospective sequential studies of the antibacterial function of neutrophils, lymphocyte responsiveness, opsonic capacity of serum and serum levels of C3(B), properdin, factor B, IgG, and albumin were made in 32 patients with severe burn injury (greater than or equal to 45%), 21 patients with severe multisystem traumatic injury, 20 high-risk, infected patients, and 22 renal transplant patients. Fifty-five episodes of bacteremia occurred in 37 of the 95 patients. Abnormal neutrophil function was clearly associated as a predisposing factor to these episodes, whereas there was no association between bacteremia and low serum levels of C3, IgG, factor B, or properdin. C3, factor B, and IgG usually rose following bacteremia as acute phase proteins, but there was evidence of a consumptive opsoninopathy in 11% of episodes. Defective opsonization was associated with a high risk of bacteremia only when there was a coexisting abnormality of neutrophil function (88% of such patients became bacteremic). None of 27 nonburned patients tested with delayed hypersensitivity antigens responded normally, and there was regularly depression of lymphocyte responsiveness to phytohemagglutinin-A and concanavalin-A in a whole blood assay related to serum immunosuppressive factors, but poor responsiveness was not associated with bacteremia.

Adolescent↗
PubMedDate not supplied

Complement in hematological neoplasias.

Total hemolytic complement (CH50) and eight antigenic fractions of the complement system were determined in 30 patients with hematological neoplasias, distributed into the following groups: six cases of non-Hodgkin's lymphomas (NHL), seven cases of chronic lymphocytic leukemia (CLL), five cases of Hodgkin's disease (HD), seven cases of acute leukemia (AL), three cases of chronic myeloid leukemia (CML) and two cases of multiple myeloma (MM). CH50 was titred accordingly to a modification of the Kabat and Mayer method, C1q, C1s, C3, C4, C5, INHC1, C3A and properdin were determined with specific antisera by Manani and Laurell's techniques. The results obtained showed significant increase in CH50 above normal values in patients with HD, AL, and CML, especially the former, even in early stages. C1s was found to be increased in CML and AL, as well as C3 in CML. C4 is increased in CML and HD. C5 follows a course similar to C4, being also increased CLL and MM. C9 is increased in all groups, except NHL. A significant increase in C3A was found in NHL, HD and AL. There were no significant variations in C1s, INHC1 and properdin in any of the former groups. No correlation was found between clinical course and complement increase. The role of complement in neoplastic disease is discussed.

Chronic Disease↗
PubMed1981

Immunofluorescence patterns in sun-exposed and not-sun-exposed skin of healthy individuals.

Immunofluorescence studies were performed on the sun-exposed (S) and not-sun-exposed (N) skin of 25 healthy volunteers regarding the incidence of the five Ig classes, albumin, fibrinogen, C1q, C4, C3B, C3c, C3d, C5, C3PA and properdin. In addition a comparative study of two so-called anti-complement antisera was made, one (CI) containing anti-(C4+C3a+C3d), the other (CII) anti-(C3B+C3c). In all biopsies the epidermal basement membrane (BM) was stained positively with anti-C3d in a fibrillar, interrupted linear, or granular pattern. A band-like picture of the BM was observed with anti-IgG , anti-albumin and anti-fibrinogen in three biopsies of the S skin. In two other S skin biopsies a lichenoid pattern was found with anti-fibrinogen, anti-C3d and IgM. The latter antiserum caused a weakly positive band-like picture of the BM, occasionally together with granular deposits, more often in the S skin (11) than in the N skin (4). In the capillary walls, granular deposits of IgM, C3d and occasionally C3c were seen in both the S skin (7) and the N skin (13). In the subjacent vessels, globular deposits positive with anti-C3d, C5 and C3c were found predominantly in the S skin. In the M. arrector pili, granular deposits of C3d and, less obviously, C5, properdin and IgM were frequently observed. A band-like, linear, or granular picture of the BM of the adnexal structures was caused by anti-C3d. IgG was found around the sweat gland in about 30%, but the other serum proteins were only rarely present. The IF patterns of the two so-called anti-complement sera showed marked differences, which were apparently caused by the presence of anti-C3d in the CI serum. The significance of these findings for the diagnostic and pathogenetic interpretation of IF results in pathology are outlined briefly.

Adult↗
PubMed1983

Neutralization of influenza virus by normal human sera: mechanisms involving antibody and complement.

All normal human sera examined neutralized WS/33 H1N1 influenza virus efficiently by one of two antibody-dependent mechanisms. A minority of the sera contained moderate levels of IgG antibody directed against the viral hemagglutinin that had the ability to directly neutralize the virus. The majority of sera tested contained very low levels of IgG anti-hemagglutinin antibody, which was detectable with a specific ELISA but not by conventional HAI assays. Such IgG antibody was unable to directly neutralize the virus. Studies with agammaglobulinemic serum and with sera depleted of and reconstituted with complement components established essential roles for IgG and the components of the classical complement pathway through C3 for neutralization. The components of the alternative and membrane attack pathways were not needed for neutralization. As anticipated from the requirement for IgG and exclusive mediation of neutralization by the classical pathway, the virus-IgG immune complex activated purified C1. Binding of C3 and C4 to the virus was demonstrated, as was classical pathway-mediated triggering of the alternative pathway, with recruitment of properdin. In addition, the H1N1 influenza virus also directly activated the alternative complement pathway in human serum, leading to C3 and properdin deposition on the viral envelope. Such direct alternative pathway activation also required immunoglobulin. However, the alternative pathway alone was unable to neutralize the virus. Thus, most normal sera examined contain low levels of IgG anti-hemagglutinin antibody, which activate the classical pathway of the complement system and neutralize WS/33 influenza virus by deposition of C3 and C4 on the viral envelope.

Agammaglobulinemia↗
PubMedDate not supplied

[The local humoral immune system in patients with malabsorption syndrome].

Content of IgA, IgG, IgM, C3 and C4 components of complement, properdin factor B, acid glycoprotein, alpha 1-antitrypsin, transferrin and ceruloplasmin were studied using immunochemical procedures in blood serum, gastric and duodenal contents of healthy persons and of patients after stomach resection. In patients with symptoms of malabsorption concentration of IgA and IgG was drastically increased in gastric and duodenal contents by 51% and 188%, respectively, and by 53% and 371%, respectively. Local activation of the complement system was also observed, which involved an increase of C3 component in stomach and small intestine by 122% and 31%, respectively. As a result of postoperative anastomositis content of albumin and the properdin factor B was distinctly increased in the duodenum. The data obtained suggest that studies of various functional proteins in gastric and duodenal contents are recommended for evaluation of a disease genesis as well as for diagnosis of acute local inflammation.

Antibody Formation↗
PubMed1996

The complement system is defective in chronic lymphatic leukemia patients and in their healthy relatives.

The present study was aimed at analyzing the existence of an impaired complement system in CLL patients. For this purpose, the serum levels of the serum complement proteins C1q, C1r, C1s, C2, C3, C4, C5, C6, C7, C8, C9, Factor B and properdin were repeatedly evaluated by means of radial immunodiffusion assay in 26 CLL patients over a period of 2 years. At the time of diagnosis, 18 of the 26 CLL patients showed low serum levels in at least one of these complement proteins as compared to a group of sex- and age-matched healthy subjects (P < 0.0001). Complement defects affected either the classical and/or the alternative pathway components, and in some case low levels of late components (C5-C9) were also observed. A reduced level of properdin was the most frequent abnormality (11/18). The presence of such abnormalities were correlated with the stage of the disease, and they were found in 100% of the patients (11) in advanced stages (Rai II-IV), and in 40% of patients (15) in early stages (0-1) (P < 0.004). Severe infections occurred in five patients; four of them were in advanced stages of the disease and had decreased levels of at least one complement component, whereas the remaining patient was in an early stage and had normal levels of complement components. These data support the notion that an impaired complement system might be involved in the pathophysiology of CLL and its infectious complications. Although more work is needed to sustain this hypothesis, we discuss the possibility on the basis of data obtained in the first-degree relatives of CLL patients, that in some CLL patients the complement deficit might reflect a genetic predisposition.

Adult↗
PubMed1997

Thrombospondin-1 suppresses tumorigenesis and angiogenesis in serum- and anchorage-independent NIH 3T3 cells.

Thrombospondin-1 (TSP1) is a multifunctional matrix protein that influences the growth and function of a variety of normal and neoplastic epithelial and mesenchymal cell types. In vivo, TSP1 has shown potent antitumor activity in suppressing tumor neovascularization. Paradoxically, however, as we have reported, NIH 3T3 fibroblasts overexpressing TSP1 acquire the transformation-associated phenotypes of serum and anchorage independence in vitro but fail to form tumors in nude mice. To investigate these divergent results, and to determine the functional domains in TSP1 that confer serum and anchorage independence as well as antitumor and antiangiogenic activities, we transfected a series of deletion constructs of TSP1 into NIH 3T3 cells and into a v-src-transformed NIH 3T3 line. The antiangiogenic activity of TSP1-expressing, v-src-transformed NIH 3T3 cells was examined by assaying the conditioned media for inhibition of endothelial cell chemotaxis and suppression of basic fibroblast growth factor-mediated angiogenesis in the rat cornea. The link between TSP1 antitumor and antiangiogenic activities was assessed by measuring the rate of tumor growth and counting factor VIII-stained microvessels in the solid tumors developing in nude mice. Our results indicate that v-src NIH 3T3 cells transfected with a 449-amino acid N-terminal domain of TSP1 exhibit a dose-dependent suppression of tumor growth and neovascularization in nude mice. Truncated forms of TSP1 containing the type 1 properdin domain suppressed both endothelial cell chemotaxis and comeal neovascularization. Furthermore, when full-length TSP1 and deletion constructs containing the antiangiogenic type I properdin domain were transfected into highly tumorigenic v-src-transformed NIH 3T3 cells, they were able to confer transdominant suppression of tumorigenicity and angiogenesis of these cells in nude mice. These results confirm the role of TSP1 as a potent inhibitor of angiogenesis and provide support for the notion that alterations in the net balance between inducers and inhibitors of angiogenesis are largely responsible for the sustained growth of solid tumors in vivo.

3T3 Cells↗
PubMed1963

Accessory plasma factors involved in the bactericidal test for type-specific antibody to group A Streptococci. II. Human plasma cofactor (s) enhancing opsonization of encapsulated organisms.

A study was made of the nature of the thermolabile plasma factors in human blood which promote the phagocytosis of Group A streptococci in vitro in the presence of optimal amounts of type-specific M antibody. The plasmas of individuals with strong opsonic activity (normal) were compared with those of some individuals whose opsonic activity was consistently weak (deficient). A general relationship was established between encapsulation of streptococci and the opsonic requirement for thermolabile plasma factor(s). Marked differences in phagocytosis of Group A organisms by human bloods were demonstrated with encapsulated strains only. Human bloods deficient in the cofactor required for opsonization of encapsulated streptococci (coopsonin) showed a normal rate of phagocytosis against all other organisms and particles studied. Furthermore, coopsonin-deficient bloods contained normal levels of four components of complement, of properdin, of lysozyme, and of direct bactericidal activity against several species of Gram-negative organisms and of E. coli bacteriophage. The independence of the streptococcal coopsonin from complement was also demonstrated by absorption of plasma with bentonite and with zymosan. Under appropriate conditions, the coopsonin was reduced without significant loss of complement. The data support the concept that the capsule of the streptococcus imposes an opsonic requirement for a plasma factor(s) which is present in varying amounts in human bloods and which appears to be independent of the complement system. The possibility that it is accessory to the latter components has not been excluded.

Antibodies↗
PubMed1978

Alternate pathway activation of complement in a Proteus mirabilis ulceration of the cornea.

A 63-year-old patient had an extensive infiltration, ulceration, and eventual perforation of the cornea caused by Proteus mirabilis. Histopathological examination of corneal tissue that was obtained at keratoplasty disclosed that the stroma was ulcerated with a diffuse infiltration of polymorphonuclear leukocytes. Immunopathological examination of the cornea revealed prominent and diffuse staining for properdin and C3 complement. There was an absence of staining for IgG, IgA, IgM, IgE, and C4 complement. These findings suggest that the alternate pathway of complement was activated in this ulcerated cornea and that immunopathological phenomena contribute to the polymorphonuclear infiltration found with Gram-negative ulcerations of the human cornea.

Complement C3↗
PubMed1986

Complement-activating abilities of defined antinuclear antibodies.

A complement-fixing immunofluorescence assay on HEp-2 cells was used to assess the ability of various antinuclear antibodies (ANA) to activate complement. Sera which contained only specific antibodies to nuclear RNP, SS-B/La, centromere, Sm antigen, double-stranded DNA, and/or nuclear histone were selected. Relative abilities of various ANA to activate complement were determined from the ratio of titers of C3, C4, or properdin-fixing ANA to the IgG ANA titers. Nuclear RNP-anti-RNP complexes activated and deposited significantly more complement C3 than other ANA (P less than 0.02). Antibodies to SS-B/La, centromere, and Sm activated more complement than anti-DNA or antihistone (P less than 0.02). Antihistone antibodies activated the least complement. These studies demonstrate that different ANA have significantly different orders of complement-activating capabilities when bound to their respective nuclear antigens.

Antibodies, Antinuclear↗
PubMed1987

Activation of the alternative pathway of complement by human serum IgA.

In order to study the activation of complement by soluble aggregates of human polyclonal serum IgA, lysis of sheep erythrocytes (E) coated with several IgA preparations was used as a model. A complement nonactivating monoclonal mouse IgG1 against IgA was used to coat the cells. IgA, isolated from normal human serum, was aggregated by either N-succinimidyl 3-(2-pyridyldithio)propionate (SPDP), glutaraldehyde, carbodiimide or heating. Depending on the size of the aggregates, and on the method of aggregation, E coated with aggregated IgA (E gamma 1.AIgA) could be lysed. The alternative pathway of complement appeared to mediate the lysis because the latter was observed in the presence of EGTA containing 5 mM Mg2+ (MgEGTA) and properdin (P) was deposited on the cells. Furthermore, no lysis was observed in C3-deficient serum. In the absence of AIgA the cells were not lysed, and no P deposition was observed. In another set of experiments E gamma 1.AIgA were first reacted with purified C3, B, D and P for 30 min at 30 degrees C, and subsequently in rat serum EDTA at 37 degrees C. Lysis occurred when E gamma 1.AIgA were prepared using SPDP-, glutaraldehyde- or carbodiimide-AIgA. Incubation of 100 micrograms/ml SPDP-AIgA with normal human serum for 30 min at 37 degrees C in the presence or absence of MgEGTA also induced consumption of total complement. The other soluble AIgA preparations were less effective in activating complement. These results suggest that polymeric serum IgA is capable of activating the alternative pathway of complement.

Complement Activation↗
PubMed1996

Complement proteins are present in developing endochondral bone and may mediate cartilage cell death and vascularization.

Normal endochondral bone formation follows a temporal sequence: immature or resting chondrocytes move away from the resting zone, proliferate, flatten, become arranged into columns, and finally become hypertrophic, disintegrate, and are replaced by bone. The mechanisms that guide this process are incompletely understood, but they include programmed cell death, a stage important in development and some disease processes. Using immunofluorescence we have studied the distribution of various complement proteins to examine the hypothesis that this sequence of events, particularly cell disintegration and matrix dissolution, are complement mediated. The results of these studies show that complement proteins C3 and Factor B are distributed uniformly in the resting and proliferating zones. Properdin is localized in the resting and hypertrophic zone but not in the proliferating zone. Complement proteins C5 and C9 are localized exclusively in the hypertrophic zones. This anatomically segregated pattern of distribution suggests that complement proteins may be important in cartilage-bone transformation and that the alternate pathway is involved.

Animals↗
PubMed1976

Symptoms and immunology of the Henoch-Schönlein-syndrome.

The Henoch-Schönlein-syndrome, or anaphylactoid purpura, is usually a childhood disease. The progess of this syndrome in adults was observed in 4 cases at our clinic. An elevated IgA level in the serum with a normal complement value seems to be of immunological interest. By immunohistological methods fibrin and IgA could be demonstrated in skin and kidney biopsies; in glomerulae C3c was observed, occasionally accompanied by Properdin-factor-B. These findings differentiate this disease from other allergic superficial vasculitids by the detection of IgG and complement; while at the same time some similarities are indicated to the so called "Berger Nephritis" with comparable kidney findings.

Adult↗