Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Properdin”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,423 records · Page 79Linked to original sources
PubMed1977

The relationship of a serum protein, C1t, to a common nonfibrillar constituent of amyloid (P component) as revealed by immunohistochemical studies.

C1t, a serum protein isolated by affinity chromatography on Sepharose bears a remarkable ultrastructural and physiocochemical resemblance to P component, a common constituent of amyloid. This study provides further evidence for their similarity by the demonstration of immunologic identity and by the presence of C1t in amyloid deposits of various types using immunoperoxidase and immunofluorescent techniques. In addition, subcomponents of C1, as well as C3, C4, C5, and properdin, were demonstrated to a limited extent. The possible role of C1t/P component in amyloidogenesis is discussed in the light of recent advances in our knowledge of the nature of amyloid substance.

Adult↗
PubMed1987

Immunohistochemical studies of reflux nephropathy. The role of extracellular matrix, membrane attack complex, and immune cells in glomerular sclerosis.

Renal tissue obtained from 8 patients with reflux nephropathy was studied by immunofluorescence with well-defined mono- and polyclonal antibodies to extracellular matrix, intermediate filament proteins, membrane attack complex of complement (MAC), and immune cells. In both unscarred and scarred areas of the tissue, intrinsic components of the human glomeruli, such as Type IV collagen, laminin, and fibronectin, accumulated in the expanded mesangium of the glomeruli. Those components were diminished or absent in the hyalinized glomeruli. Type III collagen was occasionally localized to the mesangium and synechiae in the glomeruli and was frequently observed within the nearly hyalinized glomeruli. MAC was co-deposited with C3, C5, C9, and properdin in the sclerotic area of the glomeruli, along with a decrease in the reactivity of podocytes with anti-vimentin antibody. Numerous suppressor/cytotoxic T cells were identified in the glomeruli and widened interstitum. Monocytes/macrophages also infiltrated the glomeruli. The present study suggests that accumulation of extracellular matrix, activation of the complement system, and infiltration of suppressor/cytotoxic T cells and monocytes/macrophages are closely associated with the glomerular obsolescence and the progression of reflux nephropathy.

Adolescent↗
PubMed1977

Oral pemphigus vulgaris. A report of ten cases.

The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.

Adult↗
PubMed1988

Polymeric IgA and IgA rheumatoid factor decrease the capacity of serum to solubilize circulating immune complexes in patients with primary IgA nephropathy.

Primary IgA nephropathy (IgAN) is characterized by the presence of immune complexes (IC), high levels of polymeric IgA (pIgA), and IgA rheumatoid factor (RF) in the blood. The impaired capacity of serum to solubilize IC in the presence of normal values of C hemolytic activity as well as high serum levels of C3, C4, and properdin factor B have led us to analyze whether pIgA and IgA RF from patients with IgAN where capable of inhibiting the capacity of normal human serum to solubilize immune precipitates (BSA-anti-BSA) preformed at equivalence. The results showed a significant reduced mean capacity of serum from patients with IgAN to solubilize "in vitro" immune precipitates (p less than 0.001) and significant high mean levels of pIgA (p less than 0.001) and IgA RF (p less than 0.005) in the blood. Increasing amounts of pIgA inhibited solubilization of IC in the fluid phase, and inhibitory activity was also shown by the IgA RF. There were inverse correlations between pIgA and the capacity of serum to solubilize IC (r = -0.36; p less than 0.05), and between IgA RF and the complement-mediated solubilization (r = -0.57; p less than 0.001). It is suggested that pIgA and IgA RF may be responsible for the impaired complement-mediated solubilization of serum and the persistence of insoluble nephritogenic IC in the blood of patients with primary IgAN.

Adolescent↗
PubMed1988

Hereditary C3 hypocomplementemia in the rabbit.

Hereditary hypocomplementemia of the third component of complement (C3) was found in a strain of rabbits in which hereditary C8 alpha-gamma deficiency was also found. The serum C3 concentration, haemolytic C3 activity and total complement haemolytic activity (CH50) of these animals were, respectively, 6-12%, 8-13% and 27-37% of the normal levels. The haemolytic complement activity in the C3 hypocomplementemic (C3-hypo) rabbit serum was restored in a dose-dependent manner by the addition of purified rabbit C3. The levels of factor H and properdin and components C2 and C6 were in the normal range, and the levels of factors B and D and component C8 were higher than normal. The low level of serum C3 in C3-hypo rabbits was not due to C3 conversion, partial C3 antigenicity, presence of a C3 inhibitor or hypercatabolism of normal C3. Furthermore, no change in the ratio of C3 protein levels was observed between C3-hypo and normal rabbits, even after turpentine injection. In addition, the C8 alpha-gamma deficiency condition does not affect C3 activity and C3 catabolism in vivo. Mating tests showed that the C3 hypocomplementemia is transmitted as a simple autosomal co-dominant trait. C3-hypo rabbits have a lower survival at 3 months than normal rabbits. C3-hypo rabbit serum also has a lower bactericidal activity than normal rabbit serum. The PAGE under reducing conditions showed no difference in the molecular weights of C3 alpha and C3 beta chains between C3-hypo, heterozygous and normal animals.

Aging↗
PubMed1978

[Stimulating effect of prodigiozan on nonspecific reactivity in typhoid fever, dysentery and staphylococcal infection].

A number of factors of nonspecific reactivity, i.e. complement, lysozyme, properdin, blood serum bactericidal activity, leucocyte phagocytal activity, phagocytal index and completed phagocytosis were studied on 160 guinea pigs with experimental typhoid fever, dysentery and staphylococcal infections. The data of the study showed that with the use of prodigiozan the non-specific reactivity of the host increased even within a short period of time. At the same time the pathological changes in the internal organs in their histochemical investigation decreased.

Animals↗
PubMed1986

Genetic markers in rheumatoid arthritis.

Genetic as well as environmental factors are believed to be of importance in the etiology of rheumatoid arthritis (RA). There are a number of previous studies of genetic markers in RA, but so far no genetic linkage and only a few associations have been found. Of the associations only one (with the HLA antigen DR4) appears to be well documented. In most previous association studies the patients have not been divided according to sex and family history of RA. In this investigation the HLA antigens A, B and DR and five serum protein systems (Bf, C3, Pi, Hp and Tf) were studied in patients with erosive RA, from northern Sweden. Special attention was paid to variations in the strength of associations according to sex and family history of polyarthritis. The following results were found: The frequency of the HLA antigen B27 was significantly increased in the North-Swedish population (16.6%) and among patients with a family history of polyarthritis (42.6%). In agreement with previous investigations a significantly increased frequency of the DR4 antigen was found in the RA patients. In the properdin factor B (Bf) system the S phenotype was found to be significantly increased in male patients and in patients with a family history of polyarthritis, a more severe form of RA and high titres of rheumatoid factor. No significant differences with respect to phenotype or gene frequencies were found in the C3 complement system. Thus, the association between RA and C3 found in previous investigations was not confirmed. A significant increase of rare alpha-1-antitrypsin (Pi) types (MS, MZ, MF and SZ) was found among RA patients. However, the increase concerned mainly Z heterozygotes and was more strongly pronounced among male patients. In the haptoglobin system a significant increase of the Hp2 gene and the Hp2-2 type was found among patients with a family history of polyarthritis, more pronounced among males. A significant increase of the transferrin gene C2 and of the C2 type was found among male RA patients, more pronounced among patients with a family history of polyarthritis. In 6 out of 8 gene loci studied significant associations were found, which is in agreement with a multifactorial etiology of RA.(ABSTRACT TRUNCATED AT 400 WORDS)

Arthritis, Rheumatoid↗
PubMed1986

[Investigation of HLA in patients with glioma].

HLA was studied in 35 patients with gliomas (20 anaplastic gliomas, 11 astrocytomas, 2 ependymomas, 2 oligodendrogliomas) and 38 volunteers who served as controls, by using the microdroplet lymphocyte cytotoxicity test. Phenotype frequency (PF), gene frequency (GF) and relative risk (RR) of each HLA antigen were studied statistically with the chi 2-test, where corrected P (CP) less than 0.05 was considered significant. Concerning HLA-B antigens, either Bw 61 or Bw 62 was found in 45.7% of the patients and the both in 14.3%. HLA-Bw 61 had a tendency to increase in the patients (PF = 0.46, GF = 0.27, RR = 7.16, chi 2 = 9.64, P less than 0.002, CP less than 0.064). Contrary to HLA-B antigens, HLA-DRw 6 was absent in the patient (chi 2 = 8.3379, P less than 0.004, CP less than 0.04). No relation between HLA and histological types was found. HLA has its genetic locus on the short brachium of the 6th chromosome and complement C2, C4, properdin, etc are considered to link HLA. Immunoregulatory genes (Ir genes), immunosuppressive genes (Is genes), disease susceptibility genes and disease resistant genes are also considered to link HLA, especially HLA-B, D loci. Although diseases are multifactorial, according to our report, the incidence of HLA-Bw 61 is higher, while that of HLA-DRw 6 is lower in patients with glioma. These phenomena seem very interesting considering the previous evidence that HLA has a close relation between disease susceptibility and immunological competence.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Neoplasms↗
PubMed1986

Extractable nuclear antigen (ENA) autoantibodies in SLE: an immunogenetic relationship with HLA, C4 and Bf alleles.

Sera from 36 subjects with systemic lupus erythematosus (SLE) were examined for extractable nuclear antigen (ENA) autoantibodies by immunoassay with the ribonucleoprotein (RNP) subset being determined by immunodiffusion. The prevalence of the genetic markers of HLA, the fourth complement component (C4) and properdin factor (Bf), which are all coded for within the major histocompatibility complex on chromosome 6 were analysed in relation to various parameters of these autoantibodies. The following associations were observed: The lowest ENA antibody titres of the RNP negative group were associated with HLA A9 (P less than 0.05), while the lowest RNA-ase sensitive ENA (RSE) subset antibody levels were associated with HLA Dr 1 (P less than 0.05). For the complement markers, C4 AQo was associated with the lowest affinity ENA antibodies (P less than 0.05), while the BF F allele and Fs phenotype had lower RSE antibody levels than did the S allele (P less than 0.05) and the SS phenotype (P less than 0.05) respectively. This study demonstrated diverse association between various MHC markers and ENA antibody parameters, indicating that there are distinctive immunogenetic influences over ENA autoantibodies in SLE.

Adolescent↗
PubMed1986

Immunopathology of renal disease.

The reviewed information implicates immune mechanisms in a variety of renal glomerular and tubulointerstitial diseases. Antibodies reactive with intrinsic structural or planted endogenous or exogenous antigens, and with circulating endogenous or exogenous antigens can initiate inflammatory capillary injury by localization in glomerular capillary tufts or along tubular basement membranes. This results in activation of mediator systems, including complement, neutrophils and other leukocytes, amines, peptides, and proteases, which result in vascular and tissue alterations. In some instances, nonimmune activation of complement (for instance, by the properdin system) and other mediators of vascular injury may be involved. A role of cellularly mediated immunologic injury in glomerular disease is not clear and remains the subject of considerable current research. More clearly, there is involvement of lymphocytes in tubulointerstitial and interstitial diseases as well as allograft rejection reactions. A rich armamentarium of in-vitro immunologic tests for specific antibodies, immune-complexes, serum complement levels, and renal tissue analysis provides opportunity for enhanced precision of diagnosis and monitoring progress of disease or treatment. Unfortunately, at the present time, there are not many effective therapies specific for most renal glomerular diseases; perhaps in the future better identification of offending environmental or host antigens will result in more effective prevention and treatment. Application of knowledge concerning renal glomerular diseases to the study of hypersensitivity induced tubulointerstitial injury has resulted in increasing understanding of the pathogenesis of interstitial inflammatory disease of the kidney.

Amines↗
PubMed1986

Immunogenetics of juvenile chronic arthritis in Israel.

Typing for HLA-A,B,C and DR antigens was performed in 61 Israeli patients with juvenile chronic arthritis (JCA) and in 120 unrelated controls. No significant associations were apparent in the overall patient group. DR5 was significantly increased in the non-Ashkenazi patients with pauciarticular onset of disease. The only three DRw8 positive patients in the study had pauciarticular onset. DR5 and DRw8 were found in 9 of 10 patients with age of onset less than 3 years. Increased frequencies of Bw50 and Cw6 were observed in patients with systemic onset. Typing for properdin factor (Bf) and glyoxylase (GLO) was carried out in 45 and 50 of the patients, respectively. No associations with alleles of the complement Bf system or the HLA linked GLO system were evident. The confirmation in the ethnically distinct Israeli population of the previously described association of DR5 with pauciarticular JCA suggests that this gene may be closely related to the disease susceptibility gene.

Adolescent↗
PubMed1987

A combined segregation and linkage analysis of insulin-dependent diabetes mellitus.

In an effort to clarify the mode of inheritance of insulin-dependent diabetes mellitus (IDDM), a total of 230 nuclear families with pointers were analyzed using the computer program COMBIN. Each family was ascertained without deliberate selection for multiplex families, and most families were completely typed for HLA-B, HLA-DR, and properdin factor B (Bf). There were 186 families with normal parents, 44 families with one affected parent, and no families with two affected parents. The computer program COMBIN evaluates evidence for a major locus of disease susceptibility, linkage of the major locus to a known genetic marker locus, linkage disequilibrium between the marker haplotypes and disease susceptibility, pleiotropic effects, and presence of an unlinked modifier. The parameters of COMBIN are T, Q, and D, representing the displacement, gene frequency of the IDDM allele, and dominance, respectively, of the major locus--and TM, QM, and DM being the analogous parameters of the modifier. In addition, the recombination fraction, theta, between the IDDM locus and HLA as well as the coupling frequencies are estimated. Finally, COMBIN simultaneously performs segregation and linkage analysis, with the optimal model being adjusted by the fit to the haplotype sharing distribution of IDDM. The results of these analyses indicated that the best-fitting genetic model of diabetic susceptibility appears to be a single major locus with near recessivity on a scale of standardized genetic liability, with gene frequency of the IDDM susceptibility allele of approximately 14%. In addition, the recombination fraction between the major locus and HLA is zero in all models; that is, for the B-BF-DR haplotype, the IDDM locus is tightly linked, probably (according to data from previous studies) to HLA-DR. Information determined by magnitude of coupling frequencies indicated that there is significant positive linkage disequilibrium with the haplotypes B8-BfS-DR4 and B15-BfS-DR4, significant negative linkage disequilibrium with B7-BfS-DR2, and intermediate disequilibrium for B8-BfS-DR3, B18-BfF1-DR3, and B40-BfS-DR4. Significant evidence in favor of an unlinked (to HLA) modifier (either single major locus or polygenes) could not be demonstrated. In conclusion, genetic susceptibility to IDDM appears to be most consistent with a single major locus with near recessivity that is tightly linked to HLA.

Chromosome Mapping↗
PubMed1987

[Cellular and humoral immunity and nonspecific resistance factors in chronic opisthorchiasis patients during dispensary observation].

In 116 patients with opisthorchiasis running a cholecystocholangitic variant of the disease course, the characteristics of nonspecific resistance (complement, lysozyme, properdin), cell-mediated and humoral immunity (T- and B-lymphocytes, T gamma-, T mu-, O-, D-, A-cells and auto-rosette-forming cells, IgG, IgA and IgM) have been studied. Essential changes in these characteristics before and after treatment, as well as at the remote periods of dispensary observation, have been established.

Adolescent↗
PubMed1987

Serial immunologic studies in patients with mucocutaneous lymph node syndrome (Kawasaki disease).

Twenty cases of mucocutaneous lymph node syndrome (MCLS), from Fall 1984 to April 1986, are included in this study. The serial immunoglobulin levels including IgG, IgA, IgM showed polyclonal increased immunoglobulin levels during the first month. Only one-fourth of patients had high serum IgE levels. There was no correlation with house dust mite antigen skin test and anti-mite IgE levels. By using the polyethylene glycol and ELISA methods, IgE-circulating immune complexes were detectable in 60% of high serum IgE patients. IgG-circulating immune complexes were also detected in 70% by the polyethylene glycol method and 60% by Raji cell method. CH50 and serum properdin factor B level increased during acute febrile phase. Serial T cell subset studies showed OKT4 cells decreased progressively and OKT8 levels were within normal range during the first and second week. By the third week, OKT4 cells increased and OKT8 cells decreased. This progressed to the fifth week and returned to normal range 2 months later. During the acute febrile phase of the first week, Leu2+15+ cells increased and Leu2+15- cells decreased. After the second week, the Leu2+15+ cells decreased and Leu2+15- cells increased. This increase continued to the fifth week when the Leu2+15+ cells were at the lowest level and the Leu2+15- cells at the higher level corresponding to the OKT8 change. After the fifth week, the Leu2+15+ cells and Leu2+15- cells returned to normal. Patients had increased antibody-dependent cell-mediated cytotoxic reaction at the first week of the acute febrile stage. It decreased dramatically in the second week and returned to normal 2 months later.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1979

Nutrition and the immune response -- a review.

This compacted overview of the nutrition-immune response connection underscores the role of nutrition as a deterrent to infection. Malnutrition enhances the propensity to and heightens the intensity of infections by weaknening the various host defense mechanisms. Thus: 1. Deficiencies of vitamin A, niacin, riboflavin, folic acid, vitamin B12, pyridoxine, ascorbic acid, iron and protein disrupt the tissue barriers to infection. 2. Protein-calorie, folate, iron, pyridoxine and zinc deprivations markedly depress the cell-mediated immune system. 3. Deficiencies of protein, pyridoxine, folic acid, pantothenic acid, thiamine, biotin, riboflavin, niacin-tryptophan, vitamin A and ascorbic acid inhibit humoral antibody formation in mammalian systems. 4. Vitamin A lack prevents the formation of lacrimal, salivary and sweat gland lysozymes. 5. Complement, properdin, interferon and transferrin concentrations are reduced in those nutritional deficiencies that interfere with protein synthesis. 6. Protein-calorie, iron and folate deficiencies impair phagocytosis by interfering with phagocyte microbial killing power or with phagocyte production. 7. Protein, ascorbic acid and zinc deficiencies retard wound healing that prevents spread of infectious lesions.

Animals↗
PubMed1985

Nephrotic syndrome associated with varicella infection.

A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, C1q, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.

Antigen-Antibody Complex↗
PubMed1985

T-cell subsets and response to mitogens in patients with steroid responsive nephrotic syndrome.

T-cell subsets, lymphocyte proliferative response, circulating immune complexes (CIC) and serum C3, C4 and properdin factor B were studied in 22 children suffering from steroid-responsive nephrotic syndrome (SRNS) during the acute, remission and relapse phases. Renal biopsies were performed on seven children, including three patients who relapsed after remission and all showed minimal change in their nephrotic syndrome. The lymphocyte subsets were normal in SRNS patients, but the three relapse patients had significant increase in OKT8 cells and decrease in OKT4/OKT8 ratio during both the acute and relapse phases. Use of autologous plasma will lower the lympho-proliferative response in SRNS patients during the onset of the disease and in relapse. The circulating immune complexes were detectable in seven cases during acute onset, but the serum levels of complements were normal. A review of the total data suggests that suppressor T-cell dysfunction may play a certain role in the immunopathogenesis of SRNS.

Adolescent↗
PubMed1985

IgA nephropathy in adults: immunohistologic findings and clinical course.

Laboratory examination of specimens from 123 consecutive renal biopsies performed at Victoria General Hospital, Halifax revealed six cases of mesangial deposition, predominantly of IgA, unassociated with systemic disorders. Immunohistologic examination showed deposits of only IgA in one specimen, IgA and IgG in two and IgA, IgG and IgM in three. Glomerular deposits of C3 were seen in five of the specimens, and properdin was seen in three. Glomeruli in all the specimens showed increased matrix and increased numbers of cells in the mesangium. Electron microscopy revealed deposits in the mesangium or capillary wall in all five of the specimens so studied. All six patients had proteinuria, four had microscopic hematuria, and three had hypertension; in one patient the disease progressed to renal failure.

Adult↗