An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).
A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.
Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.
A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.
The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.
A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.
The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.
A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).
A syndrome that is associated with microvascular diseases of the KIDNEY, such as RENAL CORTICAL NECROSIS. It is characterized by hemolytic anemia (ANEMIA, HEMOLYTIC); THROMBOCYTOPENIA; and ACUTE RENAL FAILURE.
Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).
A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.
Optic disk bodies composed primarily of acid mucopolysaccharides that may produce pseudopapilledema (elevation of the optic disk without associated INTRACRANIAL HYPERTENSION) and visual field deficits. Drusen may also occur in the retina (see RETINAL DRUSEN). (Miller et al., Clinical Neuro-Ophthalmology, 4th ed, p355)
C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.
Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.
A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.
A single nucleotide variation in a genetic sequence that occurs at appreciable frequency in the population.
Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
Colloid or hyaline bodies lying beneath the retinal pigment epithelium. They may occur either secondary to changes in the choroid that affect the pigment epithelium or as an autosomal dominant disorder of the retinal pigment epithelium.
The thin, highly vascular membrane covering most of the posterior of the eye between the RETINA and SCLERA.
Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.
A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. CD46 also interacts with a wide variety of pathogens and mediates immune response.
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
The proportion of one particular in the total of all ALLELES for one genetic locus in a breeding POPULATION.
A pathological process consisting of the formation of new blood vessels in the CHOROID.
A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.
The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.
The single layer of pigment-containing epithelial cells in the RETINA, situated closely to the tips (outer segments) of the RETINAL PHOTORECEPTOR CELLS. These epithelial cells are macroglia that perform essential functions for the photoreceptor cells, such as in nutrient transport, phagocytosis of the shed photoreceptor membranes, and ensuring retinal attachment.
A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.
A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.
A form of MACULAR DEGENERATION also known as dry macular degeneration marked by occurrence of a well-defined progressive lesion or atrophy in the central part of the RETINA called the MACULA LUTEA. It is distinguishable from WET MACULAR DEGENERATION in that the latter involves neovascular exudates.
Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.
The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).
Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.
Immune complex disease caused by the administration of foreign serum or serum proteins and characterized by fever, lymphadenopathy, arthralgia, and urticaria. When they are complexed to protein carriers, some drugs can also cause serum sickness when they act as haptens inducing antibody responses.
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
An individual in which both alleles at a given locus are identical.
A specific pair of human chromosomes in group A (CHROMOSOMES, HUMAN, 1-3) of the human chromosome classification.
A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).
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.
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
The genetic constitution of individuals with respect to one member of a pair of allelic genes, or sets of genes that are closely linked and tend to be inherited together such as those of the MAJOR HISTOCOMPATIBILITY COMPLEX.
GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
The regular and simultaneous occurrence in a single interbreeding population of two or more discontinuous genotypes. The concept includes differences in genotypes ranging in size from a single nucleotide site (POLYMORPHISM, SINGLE NUCLEOTIDE) to large nucleotide sequences visible at a chromosomal level.
A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.
Nonrandom association of linked genes. This is the tendency of the alleles of two separate but already linked loci to be found together more frequently than would be expected by chance alone.
Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.
The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.
A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.
Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.
A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.
A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts.
A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.
A specific pair of GROUP C CHROMOSOMES of the human chromosome classification.
The layer of pigment-containing epithelial cells in the RETINA; the CILIARY BODY; and the IRIS in the eye.
Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.
An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.
The parts of a macromolecule that directly participate in its specific combination with another molecule.
The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
An essential amino acid that is required for the production of HISTAMINE.
Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.
The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.
The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
Proteins found in any species of bacterium.
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.
Venoms from snakes of the genus Naja (family Elapidae). They contain many specific proteins that have cytotoxic, hemolytic, neurotoxic, and other properties. Like other elapid venoms, they are rich in enzymes. They include cobramines and cobralysins.
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
Individuals whose ancestral origins are in the southeastern and eastern areas of the Asian continent.
Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.
An aspect of personal behavior or lifestyle, environmental exposure, or inborn or inherited characteristic, which, on the basis of epidemiologic evidence, is known to be associated with a health-related condition considered important to prevent.
A plasma protein that circulates in increased amounts during inflammation and after tissue damage.
Genotypic differences observed among individuals in a population.
Proteins prepared by recombinant DNA technology.

Protein H, an antiphagocytic surface protein in Streptococcus pyogenes. (1/741)

Surface-associated M protein is a major virulence factor in Streptococcus pyogenes which confers bacterial resistance to phagocytosis. However, many S. pyogenes strains also express additional structurally related so-called M-like proteins. The strain studied here is of the clinically important M1 serotype and expresses two structurally related surface proteins, the M1 protein and protein H. Mutants were generated that expressed only one or none of these proteins at the bacterial surface. For survival in human blood either protein H or M1 protein was sufficient, whereas the double mutant was rapidly killed. The protein-binding properties of protein H, M1 protein, and the mutants suggest that bacterial binding of immunoglobulin G and factor H or factor H-like protein 1, which are regulatory proteins in the complement system, contribute to the antiphagocytic property.  (+info)

Multicenter trial of the quantitative BTA TRAK assay in the detection of bladder cancer. (2/741)

BACKGROUND: Human complement factor H-related protein (hCFHrp) is produced by several bladder cancer cell lines and may be useful as a cancer marker. The aim of this study was to compare urinary hCFHrp and cytology for the detection of bladder cancer found by cystoscopy in patients with suggestive signs, symptoms, or preliminary test results. METHODS: The BTA TRAK assay, a quantitative enzyme immunoassay for the bladder tumor-associated antigen in urine, was compared with exfoliative cytology in 220 patients (155 men, 65 women; mean age, 64.2 years) presenting with signs, symptoms, or preliminary diagnostic results suggestive of this disease. Cystoscopy was the standard of detection. RESULTS: In the 100 patients found to have bladder cancer, the overall sensitivities of the BTA TRAK assay (at a previously determined decision threshold of 14 kilounits/L) and cytology were 66% (66 of 100) and 33% (33 of 100), respectively (P <0.001). The BTA TRAK assay proved to be statistically more sensitive than cytology for tumor grades I and II and for stage Ta and T1 tumors. In contrast, the overall specificity of the BTA TRAK assay in the 120 patients without cystoscopically confirmed bladder cancer was 69% (83 of 120) and that of cytology was 99% (119 of 120; P <0.001). The specificity of the BTA TRAK assay was higher in patients without benign or malignant genitourinary disease other than bladder cancer (76%; n = 89) than in patients with these conditions. When the BTA TRAK assay and cytology were used together such that a positive result in either test was scored as positive and the results compared with those of the BTA TRAK assay alone, increases in overall sensitivity and equivalent specificity were observed. CONCLUSION: Because of its relatively high sensitivity, the BTA TRAK assay could complement cytology as an adjunct to cystoscopy in the diagnosis and follow-up of most patients with bladder cancer.  (+info)

In vitro analysis of complement-dependent HIV-1 cell infection using a model system. (3/741)

Previous studies based on the use of human serum as a source of C have provided evidence for the C-dependent enhancement of cell infection by HIV-1. The present study was undertaken to distinguish C from other serum factors and to identify the proteins and the mechanisms involved in C-dependent cell infection by HIV-1. The classical C activation pathway was reconstituted from the proteins C1q, C1r, C1s, C4, C2, C3, factor H, and factor I; each were purified to homogeneity. A mixture of these proteins at physiological concentrations was shown to reproduce the ability of normal human serum to enhance the infection of MT2 cells by HIV-1 at low doses of virus. This enhancing effect was abolished when heat-inactivated serum and C2- or C3-depleted serum were used, and was restored upon addition of the corresponding purified proteins. A mixture of two synthetic peptides corresponding to positions 10-15 and 90-97 of human C receptor type 2 (CD21) as well as soluble CD4 both inhibited the C-dependent infection process. These data provide unambiguous evidence that HIV-1 triggers a direct activation of the classical C pathway in vitro and thereby facilitates the infection of MT2 cells at low doses of virus. These findings are consistent with a mechanism involving increased interaction between the virus opsonized by C3b-derived fragment(s) and the CD21 cell receptors and subsequent virus entry through CD4 receptors.  (+info)

Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency. (4/741)

Factor H, a secretory glycoprotein composed of 20 short consensus repeat modules, is an inhibitor of the complement system. Previous studies of inherited factor H deficiency revealed single amino acid substitutions at conserved cysteine residues, on one allele arginine for cysteine 518 (C518R) and on the other tyrosine for cysteine 941 (C941Y) (Ault, B. H., Schmidt, B. Z., Fowler, N. L., Kashtan, C. E., Ahmed, A. E., Vogt, B. A., and Colten, H. R. (1997) J. Biol. Chem. 272, 25168-25175). To ascertain if the phenotype, impaired secretion of factor H, is due to the C518R substitution or the C941Y substitution and to ascertain the mechanism by which secretion is impaired, we studied COS-1 and HepG2 cells transfected with wild type and several mutant factor H molecules. The results showed markedly impaired secretion of both C518R and C941Y factor H as well as that of factor H molecules bearing alanine or arginine substitutions at the Cys518-Cys546 disulfide bond (C518A, C546A, C546R, C518A-C546A). In each case, mutant factor H was retained in the endoplasmic reticulum and degraded relatively slowly as compared with most other mutant secretory and membrane proteins that are retained in the endoplasmic reticulum. These data indicate that impaired secretion of the naturally occurring C518R and C941Y mutant factor H proteins is due to disruption of framework-specific disulfide bonds in factor H short consensus repeat modules.  (+info)

Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities. Italian Registry of Familial and Recurrent Hemolytic Uremic Syndrome/Thrombotic Thrombocytopenic Purpura. (5/741)

Familial hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) carry a very poor outcome and have been reported in association with decreased serum levels of the third complement component (C3). Uncontrolled consumption in the microcirculation, possibly related to genetically determined deficiency in factor H--a modulator of the alternative pathway of complement activation--may account for decreased C3 serum levels even during disease remission and may predispose to intravascular thrombosis. In a case-control study by multivariate analysis, we correlated putative predisposing conditions, including low C3 serum levels, with history of disease in 15 cases reporting one or more episodes of familial HUS and TTP, in 25 age- and gender-matched healthy controls and in 63 case-relatives and 56 control-relatives, respectively. The relationship between history of disease, low C3, and factor H abnormalities was investigated in all affected families and in 17 controls. Seventy-three percent of cases compared with 16% of controls (P < 0.001), and 24% of case-relatives compared with 5% of control-relatives (P = 0.005) had decreased C3 serum levels. At multivariate analysis, C3 serum level was the only parameter associated with the disease within affected families (P = 0.02) and in the overall study population (P = 0.01). Thus, subjects with decreased C3 serum levels had a relative risk of HUS or TTP of 16.56 (95% confidence interval [CI], 1.66 to 162.39) within families and of 27.77 (95% CI, 2.44 to 314.19) in the overall population, compared to subjects with normal serum levels. Factor H abnormalities were found in four of the cases, compared with three of the healthy family members (P = 0.02) and none of the controls (P = 0.04) and, within families, factor H abnormalities were correlated with C3 reduction (P < 0.05). Reduced C3 clusters in familial HUS and TTP is likely related to a genetically determined deficiency in factor H and may predispose to the disease. Its demonstration may help identify subjects at risk in affected families.  (+info)

Familial relapsing haemolytic uraemic syndrome and complement factor H deficiency. (6/741)

BACKGROUND: In a recent study of three families we have found that inherited haemolytic uraemic syndrome (HUS) maps to a region of chromosome 1q containing the gene for complement factor H. In one of these families and also in a case of sporadic D-HUS, we have identified mutations in the factor H gene. A further family with inherited HUS has therefore been investigated. METHODS: DNA extracted from the family members and DNA extracted from archival post-mortem material from a deceased family member, was studied. Review of renal biopsies and study of complement components was also undertaken. RESULTS: This family demonstrates an inherited deficiency of complement factor H. Non-diarrhoeal HUS has affected at least two family members with half normal levels of factor H. CONCLUSION: These findings represent further evidence of the association between factor H dysfunction and HUS.  (+info)

Identification of human complement Factor H as a ligand for L-selectin. (7/741)

The selectin family of adhesion molecules (E-, P- and L-selectins) is involved in leukocyte recruitment to sites of inflammation and tissue damage. Recently it has been shown that L-selectin is involved not only in leukocyte tethering and rolling, but also plays an important role in leukocyte activation. For example, glycosylation-dependent cell-adhesion molecule 1 (GlyCAM-1), a known ligand for L-selectin, has been shown to enhance beta2-integrin function. GlyCAM-1 is a secreted protein and is present in mouse serum at a concentration of approx. 1.5 microg/ml. There is no obvious GlyCAM-1 homologue in man and, to date, L-selectin ligand(s) from human serum have not been characterized. Therefore we have used L-selectin affinity chromatography, followed by ion-exchange chromatography, to isolate specific ligand(s) for L-selectin. Using this procedure, we have isolated three major glycoproteins of apparent molecular masses 170 kDa, 70kDa and 50 kDa. The 170 kDa protein band was digested with trypsin and peptides were analysed by delayed extraction matrix-assisted laser desorption ionization MS and protein database searching. The 170 kDa protein was identified as the human complement protein Factor H. Human Factor H, isolated by a different method, was shown to bind specifically to L-selectin in the presence of CaCl2, and binding was inhibited by anti-L-selectin antibodies, fucoidan and lipopolysaccharide. Only a part of the purified Factor H preparation bound to immobilized L-selectin. The interaction of Factor H with leukocyte L-selectin was shown to induce the secretion of tumour necrosis factor-alpha (TNF-alpha). Pretreatment of Factor H with sialidase reduced both the binding of L-selectin to Factor H and the Factor H-induced L-selectin-mediated TNF-alpha secretion by leukocytes. Taken together, these results demonstrate that a post-translationally modified form of human plasma Factor H is a potential physiological ligand for L-selectin.  (+info)

Resistance to both complement activation and phagocytosis in type 3 pneumococci is mediated by the binding of complement regulatory protein factor H. (8/741)

To study the role of surface-associated proteins in the virulence of Streptococcus pneumoniae, we used two serotype 3 strains, ATCC 6303 and WU2, and two PspA-negative mutants of WU2, an encapsulated one, JY1123 (Caps(+)/PspA(-)), and an unencapsulated one, DW3.8 (Caps(-)/PspA(-)). ATCC 6303 and WU2 were highly virulent in mice, while the virulence of JY1123 was slightly decreased (50% lethal doses [LD(50)s], 24, 6, and 147 CFU/mouse, respectively); DW3.8 was avirulent (LD(50), 2 x 10(8) CFU). In vitro, ATCC 6303, WU2, and JY1123 (Caps(+)/PspA(-)) strongly resisted complement activation and complement-dependent opsonophagocytosis, whereas DW3.8 (Caps(-)/PspA(-)) was easily phagocytized in fresh serum. Trypsin treatment of ATCC 6303, WU2, and JY1123 (Caps(+)/PspA(-)) resulted in enhanced complement activation and complement-dependent opsonophagocytosis. Trypsin had no deleterious effect on the polysaccharide capsule. In addition, trypsin pretreatment of ATCC 6303 strongly reduced virulence upon intraperitoneal challenge in mice. This indicated that surface proteins play a role in the resistance to complement activation and opsonophagocytosis and contribute to the virulence of type 3 pneumococci. In subsequent experiments, we could show that the modulation of complement activation was associated with surface components that bind complement regulator factor H; binding is trypsin sensitive and independent of prior complement activation. Immunoblotting of cell wall proteins of the virulent strain ATCC 6303 with anti-human factor H antibody revealed three factor H-binding proteins of 88, 150, and 196 kDa. Immunogold electron microscopy showed a close association of factor H-binding components with the outer surface of the cell wall. The role of these factor H-binding surface proteins in the virulence of pneumococci is interesting and warrants further investigation.  (+info)

Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation in mice.
4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type
Purpose: Several key components of the complement cascade are associated with the pathogenesis of age-related macular degeneration (AMD), including the Y402H variation in the gene encoding complement factor H (CFH), which confers a several-fold increased risk for developing AMD. Earlier studies, including ours, demonstrated an association between drusen, a hallmark of early AMD, and increased inflammatory cytokines in retina of postmortem eye tissues. In this study, we address the correlation between plasma cytokines and drusen load, choroidal thickness and the Y402H polymorphism in CFH gene in patients with dry AMD.. Methods: Forty-four patients with dry AMD were recruited. Drusen area and volume were determined using the spectral-domain optical coherence tomography (SD-OCT) software, and choroidal thickness was measured using enhanced depth imaging (EDI). The subjects blood samples were analyzed for multiple cytokines using Bio-Plex suspension assays and genotyped for the CFH Y402H ...
Purpose: : Previous studies have demonstrated that the Y402H polymorphism in the complement factor H (CFH) gene is associated with an approximately 3-fold increased risk for age-related macular degeneration (AMD) in Caucasians of predominantly European descent. The prevalence of AMD varies widely among persons of different ethnicities. In this study we sought to determine the frequency of this polymorphism in control populations of Caucasians, African Americans, Hispanics, Somalis, and Japanese. Moreover, we investigated whether specific AMD phenotypes are associated with this polymorphism. Methods: : Normal control populations were assembled for each ethnic group: Caucasian (n=148), Somali (n=128), African American (n=75), Hispanic (n=100), and Japanese (n=82). Individuals were genotyped using NIaIII restriction digestion. The frequency of the histidine (His) allele at position 402 of the CFH gene was determined. A bioinformatic approach was used to identify single nucleotide polymorphisms in ...
TY - JOUR. T1 - Synthesis of complement factor H by retinal pigment epithelial cells is down-regulated by oxidized photoreceptor outer segments. AU - Chen, Mei. AU - Forrester, John Vincent. AU - Xu, Heping. PY - 2007/4. Y1 - 2007/4. N2 - Complement activation is thought to be involved in the pathogenesis of age-related macular degeneration (AMD), in part because certain gene polymorphisms in complement factor H (CFH), an important regulator of the alternative complement activation pathway, are high risk factors for AMD. How CFH is regulated locally at the retina/choroid interface and how this contributes to AMD development remain unknown. In the present study, we have confirmed that CFH was detectable by immunohistochemistry in the choroid, and at low levels in the RPE cell and interphotoreceptor matrix, but appeared to be concentrated in dense patches in Bruchs membrane. In vitro, cultured human and mouse RPE cells expressed high levels of CFH as evidenced by immunohistochemistry and western ...
SR GROUP - Exporter, Importer, Manufacturer, Distributor, Supplier, Trading Company of Rat CFH(Complement Factor H) ELISA Kit based in Delhi, India
Complement factor H (CFH) protein is an inhibitor of the alternative pathway of complement (AP) both in the fluid phase and on the surface of host cells. Mouse and human complement factor H-related (CFHR) proteins also belong to the fH family of plasma glycoproteins. The main goal of the current study was to compare the presence of mRNA for two mCFHR proteins in spontaneously developing autoimmune diseases in mice such as dense deposit disease (DDD), diabetes mellitus (DM), basal laminar deposits (BLD), collagen antibody-induced arthrits (CAIA) and systemic lupus erythematosus (SLE). Here we report for the first time that the CFHR-C mRNA was universally absent in the liver from three strains of lupus-prone mice and in a diabetic-prone mouse strain. The mRNA levels (pg/ng) for CFH and CFHR-B in MRL-lpr/lpr, at 9wks and 23wks were 707.2±44.4, 54.5±5.75 and 729±252.9, 74.04±22.76, respectively. The mRNA levels for CFH and CFHR-B in NZB/NZW mice, at 9wks and 54wks were 579.9±23.8, 58.8±1.41 ...
Nontypeable Haemophilus influenzae (NTHi) cause a range of illnesses including otitis media, sinusitis, and exacerbation of chronic obstructive pulmonary disease, infections that contribute to the problem of antibiotic resistance and are themselves often intractable to standard antibiotic treatment regimens. We investigated a strategy to exploit binding of the complement inhibitor Factor H (FH) to NTHi as a functional target for an immunotherapeutic containing the NTHi binding domain of FH fused to the Fc domain of IgG1. Chimeric proteins containing the regions that most FH-binding bacteria use to engage human FH, domains 6 and 7 (FH6,7/Fc) and/or 18 through 20 (FH18-20/Fc), were evaluated for binding to NTHi. FH6,7/Fc bound strongly to each of seven NTHi clinical isolates tested and efficiently promoted complement-mediated killing by normal human serum. FH18-20/Fc bound weakly to three of the strains but did not promote complement dependent killing. Outer-membrane protein P5 has been implicated in FH
Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent studies have identified a factor H-associated form of HUS, caused by gene mutations that cluster in the C-terminal region of the complement regulator factor H. Here we report how three mutations (E1172Stop, R1210C, and R1215G; each of the latter two identified in three independent cases from different, unrelated families) affect protein function. All three mutations cause reduced binding to the central complement component C3b/C3d to heparin, as well as to endothelial cells. These defective features of the mutant factor H proteins explain progression of endothelial cell and microvascular damage in factor H-associated genetic HUS and indicate a protective role of factor H for tissue integrity during thrombus formation.. ...
Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent studies have identified a factor H-associated form of HUS, caused by gene mutations that cluster in the C-terminal region of the complement regulator factor H. Here we report how three mutations (E1172Stop, R1210C, and R1215G; each of the latter two identified in three independent cases from different, unrelated families) affect protein function. All three mutations cause reduced binding to the central complement component C3b/C3d to heparin, as well as to endothelial cells. These defective features of the mutant factor H proteins explain progression of endothelial cell and microvascular damage in factor H-associated genetic HUS and indicate a protective role of factor H for tissue integrity during thrombus formation.. ...
The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
SEA635Mu, CF-H; FH; FHL1; ARMD4; ARMS1; CFHL3; HF1; HF2; HUS; H Factor 2; Age-Related Maculopathy Susceptibility 1; Adrenomedullin binding protein | Products for research use only!
Eculizumab: This humanized monoclonal antibody against complement C5 ultimately prevents the formation of C5-9 membrane attack complex and generation of prothrombotic C5a.. Plasma exchange: Plasma exchange can be used to remove the mutated protein and replace them with normal proteins in patients who do not respond to Eculizumab. Transplant patients with auto-antibodies against complement factor H have been successfully treated with plasma- exchange, rituximab and high dose steroids.. Liver- kidney transplantation: There have been some case reports of combined liver and kidney transplantation, which has been successful in treating HUS in patients with complement factor H mutation.. It is important to identify the gene mutation that led to atypical HUS in every patient who is evaluated for kidney transplantation and has a history of atypical HUS, in order to estimate the risk of recurrence and plan eventual prevention therapy. The knowledge of the mutation would also allow appropriate ...
Nita Amornsiripanitch1, Shaolin Hong1, Michael J. Campa1, Michael M. Frank2, Elizabeth B. Gottlin1, and Edward F. Patz Jr.1,3 Author Affiliations. Corresponding Author: Edward F. Patz, Jr., Duke University Medical Center, Department of Radiology, Box 3808, Durham, NC 27710. Phone: 919-684-7311; Fax: 919-681-7165; E-mail: [email protected] ...
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Being a major first line of immune defense, the complement system keeps a constant vigil against viruses. Its ability to recognize a large panoply of viruses and virus-infected cells, and trigger the effector pathways, results in neutralization of viruses and killing of the infected cells. This selection pressure exerted by complement on viruses has made them evolve a multitude of countermeasures. These include targeting the recognition molecules for avoidance of detection, targeting key enzymes and complexes of the complement pathways like C3 convertases and C5b-9 formation - either by encoding complement regulators or by recruiting membrane-bound and soluble host complement regulators, cleaving complement proteins by encoding protease, and inhibiting the synthesis of complement proteins. Additionally, viruses also exploit the complement system for their own benefit. For example, they use complement receptors as well as membrane regulators for cellular entry as well as their spread. Here, we provide an
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The role of cell membrane-associated human factor H for the binding of cell-bound C3b to complement receptor-carrying (CR+) cells was investigated. Pretreatment of CR+ cells with antibodies to factor H inhibited the adherence of C3b-coated red cells to human tonsil lymphocytes (TL) and peripheral bl …
Complement inhibiting surface proteins of pathogenic bacteria provide candidates for vaccines because of two reasons. First, an immune response against them would recognize the microbes and secondly, it would neutralize the key bacterial virulence mechanism. Prerequisites for a vaccine protein include the following: (i) it should show limited variability, (ii) it should be immunogenic and the immune response against it should cover a sufficiently broad range of microbial strains, (iii) it should not be hidden beneath a capsule, long LPS O-polysaccharide side chains or a protein coat and (iv) it should not raise unwanted immune responses against host structures. Bacterial complement inhibitors often act by binding the soluble inhibitors factor H or C4 bp, by blocking C3 or C5 activation or by enzymatically cleaving key complement components. Inhibitors have been found from all major types of pathogens and may offer promise as rational vaccine candidates for preventing diseases such as ...
Progress in Understanding AMD Genetics. While a number of environmental and dietary risk factors have been linked with AMD, progress in identifying genetic components associated with the disease has been somewhat slower. However significant progress has been made over the last few years: up to six regions of the genome have been identified as potentially harboring AMD genes.. One region repeatedly linked to AMD in family based studies is located on chromosome 1. This year, several independent research teams reported a strong association of AMD with a chromosomal 1 gene that encodes the protein, complement factor H (CFH). A single base change in the CFH gene introduces a single amino acid change into the sequence of the variant CFH protein it produces, replacing the amino acid tyrosine with histidine. Understanding this simple genetic alteration - a change in a single unit of DNA out of the 3 billion or so units that make up our genetic blueprint - could hold future promise for those at risk for ...
1: AMD and complement factor H (CFH). Common sequence variants of CFH have major roles in determining susceptibility to age-related macular degeneration (AMD)....
Complement, C3 Convertase, Regulation, Cells, Disease, Complement Factor H, Inhibition, Therapeutic, Transplant, Allograft, Donor, Donors, and Graft
Hypothetical LOC387715 is a second major susceptibility gene for age-related macular degeneration, contributing independently of complement factor H to disease risk. ...
In the case of AMD, Erica Fletcher et al. have interestingly reviewed the new means of detecting the signs of early-stage disease.[46] , [47] AMD has genetic and environmental risk factors. Genetic testing is now readily available using a combination of 16 genes to help predict the risk profile of an individual. Among the genes associated with an increased risk of developing AMD is complement factor H (CFH), the mutations of which contribute to explain immune dysregulation in AMD. Considering retinal imaging, Hogg et al. have recently highlighted that a particular form of drusens called reticular pseudo-drusen, at a subretinal level, is a risk-factor for progression to late-stage disease. [48] A research team in the Netherlands has developed a software to analyze color fundus photographs so as to quantify and characterize drusens and determine a risk assessment. Using fundus autofluorescence (FAF), the RPE cell dysfunction can be detected through the accumulation of lipofuscin. Some scientists ...
T Cells, Mice, Phenotype, Regulatory T Cells, Cell, Cells, Muscle, Myopathies, Myopathy, Patients, Complement Factor H, Animals, Memory, Administration, Blood, Bone, Bone Marrow, Bone Marrow Transplantation, Cell Numbers, Cell Transplantation
The PDB archive contains information about experimentally-determined structures of proteins, nucleic acids, and complex assemblies. As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.
Background: Complement mediated neuroinflammation is implicated in several neurodegenerative diseases. Complement 3 (C3) plays a key role in the complement cascade as its proteolytic fragments stimulate proinflammatory responses. Complementary factor H (CFH) regulates C3 activity and it can alter immune complex mediated neuroinflammatory responses. We hypothesized that loss of CFH function due to presence of a missense rs534399-G/T polymorphism (Val1007Leu codon change) will impair complement regulation leading to immune complex deposition, neuroinflammatory responses and neurocognitive impairment in HIV-infected adults.. Methods: 562 subjects from the CHARTER cohort were evaluated by logistic regression for association of CFH polymorphisms and clinical outcomes at baseline and longitudinally. Global impairment, inclusion review, race, sex, age, AIDS, CD4, nadir CD4, undetectable plasma/CSF, and antiretroviral status were included in the cross-sectional analysis. Functions between genes and ...
This study explores the relationship between defined antigens of the C3 molecule and those surface structures that are involved in interaction with factors I and H. Methylamine treatment at pH 11 followed by neutralization converts C3 into a modified state in which the molecule is optimally susceptible to cleavage by factor I in the presence of factor H. The modified C3 is characterized by an antigenic profile with expression of antigens of the C3(N), C3(S), and C3(D) subsets. These antigenic properties closely mirror those of physiologically bound C3b, suggesting that modification of antigenic expression upon denaturation of C3 reflects a regulatory mechanism for I and H function. Immunochemical studies of the alkaline-denatured C3 suggested that factor H interacts with surfaces of C3 that are situated within the C3c fragment and that are defined by C3(SN) antigens, while factor I predominantly interacts with C3(SN) antigens associated with the C3d fragment and with C3(D) antigens hidden in ...
The complement system labels microbes and host debris for clearance. Degradation of surface-bound C3b is pivotal to direct immune responses and protect host cells. How the serine protease factor I (FI), assisted by regulators, cleaves either two or three distant peptide bonds in the CUB domain of C3b remains unclear. We present a crystal structure of C3b in complex with FI and regulator factor H (FH; domains 1-4 with 19-20). FI binds C3b-FH between FH domains 2 and 3 and a reoriented C3b C-terminal domain and docks onto the first scissile bond, while stabilizing its catalytic domain for proteolytic activity ...
References for Abcams Recombinant Human FH protein (ab116496). Please let us know if you have used this product in your publication
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This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. This protein co-localizes with C3, binds C3b in a dose-dependent manner, and is recruited to tissues damaged by C-reactive protein. Allelic variations in this gene have been associated, but not causally linked, with two different forms of kidney disease: membranoproliferative glomerulonephritis type II (MPGNII) and hemolytic uraemic syndrome (HUS). [provided by RefSeq, Jan 2010 ...
Membranoproliferative Glomerulonephritis type II {1:Abrera-Abeleda et al. (2006)} summarized features of MPGN relevant to the complement cascade. MPGN type II, also known as dense deposit disease, causes chronic renal dysfunction that progresses to end-stage renal disease in about half of patients within 10 years of diagnosis. MPGN types I and III are variants of immune complex-mediated disease; MPGN II, in contrast, has no known association with immune complexes ({2:Appel et al., 2005}). MPGN II accounts for less than 20% of cases of MPGN in children and only a fractional percentage of cases in adults. Both sexes are affected equally, with the diagnosis usually made in children between the ages of 5 and 15 years who present with nonspecific findings such as hematuria, proteinuria, acute nephritic syndrome, or nephrotic syndrome. More than 80% of patients with MPGN II are positive for serum C3 nephritic factor (C3NeF), an autoantibody directed against C3bBb, the convertase of the alternative ...
Factor H-related protein 1 (FHR-1): A complement regulatory protein and guardian of necrotic type surfaces.: Factor H-related protein 1 (FHR-1) is a member of t
To further investigate this hypothesis, we enrolled group 2. In group 2, we found individuals with the rs6677604-A allele had increased CFH levels. Recently, Ansari et al.12 reported that rs6677604 and CFHR3-1∆ were strongly correlated with plasma CFH concentration, which is in accordance with our findings. Moreover, the CFHR3-1∆ resulted in the absence of CFHR1 protein, which was recently shown to function as a competitive antagonist of CFH.33 Therefore, higher CFH levels, together with the absence of antagonist (CFHR1 protein), resulted in the robust complement inhibition, which is represented by the higher circulating C3 and lower C3a that we observed in patients with IgAN with the rs6677604-AA genotype and CFHR3-1Δ. In accordance with our findings, Yang et al.34 reported that rs3753394 in CFH was associated with circulating C3 levels. Because rs3753394 and rs6677604 are in LD, the study by Yang et al.34 provides independent validation for our findings. Meanwhile, by showing that plasma ...
Persistent pathogens have evolved to avoid elimination by the mammalian immune system including mechanisms to evade complement. Infections with African trypanosomes can persist for years and cause human and animal disease throughout sub-Saharan Africa. It is not known how trypanosomes limit the action of the alternative complement pathway. Here we identify an African trypanosome receptor for mammalian factor H, a negative regulator of the alternative pathway. Structural studies show how the receptor binds ligand, leaving inhibitory domains of factor H free to inactivate complement C3b deposited on the trypanosome surface. Receptor expression is highest in developmental stages transmitted to the tsetse fly vector and those exposed to blood meals in the tsetse gut. Receptor gene deletion reduced tsetse infection, identifying this receptor as a virulence factor for transmission. This demonstrates how a pathogen evolved a molecular mechanism to increase transmission to an insect vector by exploitation of a
TY - JOUR. T1 - First-line therapy in atypical hemolytic uremic syndrome. T2 - consideration on infants with a poor prognosis. AU - Szarvas, Nóra. AU - Szilágyi, Ágnes. AU - Tasic, Velibor. AU - Nushi-Stavileci, Valbona. AU - Sofijanova, Aspazija. AU - Gucev, Zoran. AU - Szabó, Miklós. AU - Szabó, Attila. AU - Szeifert, Lilla. AU - Reusz, György. AU - Rusai, Krisztina. AU - Arbeiter, Klaus. AU - Müller, Thomas. AU - Prohászka, Zoltán. PY - 2014/12/11. Y1 - 2014/12/11. N2 - BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare and heterogeneous disorder. The first line treatment of aHUS is plasma therapy, but in the past few years, the recommendations have changed greatly with the advent of eculizumab, a humanized monoclonal anti C5-antibody. Although recent recommendations suggest using it as a primary treatment for aHUS, important questions have arisen about the necessity of immediate use of eculizumab in all cases. We aimed to draw attention to a specific subgroup of aHUS ...
ConclusionThe atypical hemolytic uremic syndrome is a rare disease entity requiring a high index of suspicion to diagnose. It is a diagnosis of exclusion. Early diagnosis with prompt treatment will render a better outcome. The atypical hemolytic uremic syndrome needs to be considered in all patients with thrombotic microangiopathy....
The complement system is an essential component of the innate and acquired immune system, and consists of a series of proteolytic cascades that are initiated by the presence of microorganisms. In health, activation of complement is precisely controlled through membrane-bound and soluble plasma-regulatory proteins including complement factor H (fH; ref. 2), a 155 kDa protein composed of 20 domains (termed complement control protein repeats). Many pathogens have evolved the ability to avoid immune-killing by recruiting host complement regulators and several pathogens have adapted to avoid complement-mediated killing by sequestering fH to their surface. Here we present the structure of a complement regulator in complex with its pathogen surface-protein ligand. This reveals how the important human pathogen Neisseria meningitidis subverts immune responses by mimicking the host, using protein instead of charged-carbohydrate chemistry to recruit the host complement regulator, fH. The structure also indicates
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. In most cases it is caused by chronic, uncontrolled activation of the complement system, a branch of the bodys immune system that destroys and removes foreign particles. The disease affects both children and adults and is characterized by systemic thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to stroke, heart attack, kidney failure, and death. The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane cofactor protein), or is occasionally due to acquired neutralizing autoantibody inhibitors of these complement system components, for example anti-factor H antibodies. Despite the use of supportive care, historically an estimated 33-40% of patients died or developed end-stage renal disease (ESRD) with the first ...
C3 glomerulopathy defines a subgroup of membranoproliferative glomerulonephritis (MPGN) characterized by complement 3 (C3)-positive, immunoglobulin-negative deposits in immunofluorescence microscopy. It comprises 3 clinical conditions: dense deposit disease, C3 glomerulonephritis, and complement factor H-related 5 (CFHR5) nephropathy. Mutations in genes encoding regulatory proteins of the alternative complement pathway have been described. A 16-year-old girl was admitted to the hospital due to periorbital edema. Nephrotic syndrome accompanied by low C3 level was diagnosed. Renal biopsy showed MPGN in light microscopy, only C3 deposits in immunofluorescence microscopy, and subendothelial electron dense deposits and capillary basement membrane thickening with double contour formation in electron microscopy. C3 nephritic factor and anti complement factor H antibody were negative. Complement factor H level was normal. Genetic screening showed a novel heterozygous p.Cys269Arg variation in the CFHR5 ...
The opportunistic human pathogenic fungus Aspergillus fumigatus is a major cause of fungal infections in immunocompromised patients. Innate immunity plays an important role in the defense against infections. The complement system represents an essential part of the innate immune system. This cascade system is activated on the surface of A. fumigatus conidia and hyphae and enhances phagocytosis of conidia. A. fumigatus conidia but not hyphae bind to their surface host complement regulators factor H, FHL-1, and CFHR1, which control complement activation. Here, we show that A. fumigatus hyphae possess an additional endogenous activity to control complement activation. A. fumigatus culture supernatant efficiently cleaved complement components C3, C4, C5, and C1q as well as immunoglobulin G. Secretome analysis and protease inhibitor studies identified the secreted alkaline protease Alp1, which is present in large amounts in the culture supernatant, as the central molecule responsible for this ...
The short consensus repeat domain (SCR, complement control protein module, sushi-domain) is a structural unit found in multiple adjacent copies in more than 40 human proteins. Each bead-like domain is composed of approximately 60 residues and the adjacent domains are connected in a head-to-tail fash …
Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins. Thus, aHUS provides an archetypal complement-mediated disease with which to model new therapeutic strategies and treatments. Herein, we show that, when transferred to mice, an aHUS-associated gain-of-function change (D1115N) to the complement-activation protein C3 results in aHUS. Homozygous C3 p.D1115N (C3KI) mice developed spontaneous chronic thrombotic microangiopathy together with hematuria, thrombocytopenia, elevated creatinine, and evidence of hemolysis. Mice with active disease had reduced plasma C3 with C3 fragment and C9 deposition within the kidney. Therapeutic blockade or genetic deletion of C5, a protein downstream of C3 in the complement cascade, protected homozygous C3KI mice from thrombotic microangiopathy and aHUS. Thus, our data provide in vivo modeling evidence that ...
The complement system plays an important role in our innate immune system. Complement activation results in clearance of pathogens, immune complex, and apoptotic cells. The host is protected from complement-mediated damage by several complement regulators. Factor H (FH) is the most important fluid-phase regulator of the alternative pathway of the complement system. Heterozygous mutations in FH are associated with complement-related diseases such as atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration. We recently described an agonistic anti-FH mAb that can potentiate the regulatory function of FH. This Ab could serve as a potential new drug for aHUS patients and alternative to C5 blockade by eculizumab. However, it is unclear whether this Ab can potentiate FH mutant variants in addition to wild-type (WT) FH. In this study, the functionality and potential of the agonistic Ab in the context of pathogenic aHUS-related FH mutant proteins was investigated. The binding ...
The complement system plays an important role in our innate immune system. Complement activation results in clearance of pathogens, immune complex, and apoptotic cells. The host is protected from complement-mediated damage by several complement regulators. Factor H (FH) is the most important fluid-phase regulator of the alternative pathway of the complement system. Heterozygous mutations in FH are associated with complement-related diseases such as atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration. We recently described an agonistic anti-FH mAb that can potentiate the regulatory function of FH. This Ab could serve as a potential new drug for aHUS patients and alternative to C5 blockade by eculizumab. However, it is unclear whether this Ab can potentiate FH mutant variants in addition to wild-type (WT) FH. In this study, the functionality and potential of the agonistic Ab in the context of pathogenic aHUS-related FH mutant proteins was investigated. The binding ...
This is a Phase 3, multicenter study of OMS721 in adults and adolescents with atypical hemolytic uremic syndrome (aHUS). The uncontrolled, open-label study will evaluate the effect of OMS721 in subjects with plasma therapy-resistant aHUS and plasma therapy-responsive aHUS. This study has four periods: Screening, Treatment Induction, Treatment Maintenance, and Follow-up. Approximate enrollment is 80 subjects. An interim analysis will be performed after 40 subjects have completed 26 weeks of treatment for potential registration ...
Abstract. Abstract 2085Introduction:. Atypical hemolytic uremic syndrome (aHUS) is a rare chronic disorder characterized by persistent uncontrolled complement
Neonatal atypical hemolytic uremic syndrome is characterized by hemolytic anemia, thrombocytopenia and thrombotic microangiopathy. Disease caused by a..
Human umbilical vein endothelial cells grown in vitro under standard conditions contain a high level of mRNA specific for the complement regulatory factors H and I. An additional 1.8-kb mRNA encoding a truncated form of factor H is also present. IFN-gamma stimulation of the cells causes a 6-7 fold increase in both factor H mRNA species, and a greater than 10-fold increase in factor I mRNA. IL-1 and LPS slightly suppressed factor H mRNA, while TNF had no effect. mRNA for factor B is also detectable in IFN-gamma-stimulated cells, but messengers for C1q, C4bp, and CR3 beta chain were not found. Secretion of factor H protein was also stimulated by IFN-gamma. The presence of mRNA for factors H, B, and I, together with C3 secretion, demonstrated by others, suggests that endothelial cells can assemble the complete alternative complement pathway. Endothelial cell complement may be involved in leukocyte-endothelium interactions mediated by leukocyte C3 receptors. ...
Zuber J, Le Quintrec M, Krid S, Bertoye C, Gueutin V, Lahoche A et al (2012) Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant 12(12):3337-3354. doi:10.1111/j.1600-6143.2012.04252.x CrossRefPubMedGoogle Scholar ...
Dense deposit disease (DDD) is a condition that primarily affects kidney function. Signs and symptoms usually start between the ages of 5 and 15 but may also begin in adulthood. The major features of DDD are due to kidney malfunction, and often include proteinuria; hematuria; reduced amounts of urine; low levels of protein in the blood; and swelling in many areas of the body. About half of affected people develop end-stage renal disease (ESRD) within 10 years after symptoms start. DDD can have genetic or non-genetic causes. It can be caused by mutations in the C3 and CFH genes; it may develop as a result of both genetic risk factors and environmental triggers; or it can result from the presence of autoantibodies that block the activity of proteins needed for the bodys immune response. Most cases are sporadic (occurring by chance in people with no history of the disorder in their family ...
Age related macular degeneration (AMD) is one of the leading causes of blindness in the elderly worldwide. Due to earlier clinical observations that AMD concentrates in families, a genetic component to the disease has been suggested. Several genetic studies have identified areas of association with AMD on chromosomes 1q31 and 10q26. On chromosome 1q31 the most famous single nucleotide polymorphism (SNP) to be linked to AMD was rs1061170 on the complement factor H gene (CFH). This SNP was studied in several populations and was found to be highly associated with AMD in most Caucasian populations with odds Ratio (OR) reaching 9.79 for CC homozygous and 4.36 for CT heterozygous in some cases. However in Japan, China, Korea and South Africa no or weak association was found. Other SNPs on chromosome 10q26 were also associated with AMD. SNP rs10490924 (Ala69Ser) on the age related maculopathy susceptibility 2 gene (ARMS2) was associated with AMD in the USA, Germany, China, Turkey and India with OR values
An example within the aHUS group demonstrates how unique, pathology-related factors influence the treatment of aHUS in these relative settings. As mentioned previously, at the genetic level, a CFH mutation may be transcribed into a nonfunctional protein leading to aHUS. However, loss of factor H function can also be the consequence of autoantibodies to this protein. Although the phenotypic consequence is dysregulation of the alternate pathway of complement and aHUS, different targeted treatments are possible because the pathophysiology is fundamentally different. Certainly, anticomplement therapy appears to work for both types of aHUS. However, in patients with autoimmune-mediated HUS, a more direct therapy would target the antibody and not the terminal complement pathway. This tenet is the basis for considering anticellular immune suppression to reduce antibody titer and limit risk for exacerbation without compromising complement activity in this select patient cohort.. The study by Chinchilla ...
There is increasing evidence that human complement factor H-related protein 1 (CFHR1) plays a crucial role in the development of malignant diseases. However, few studies have identified the roles of CFHR1 in the occurrence and prognosis of lung adenocarcinoma (LADC). In the present study, comprehensive bioinformatic analyses of data obtained from the Oncomine platform, UALCAN and Gene Expression Profiling Interactive Analysis (GEPIA) demonstrated that CFHR1 expression is significantly reduced in both LADC tissues and cancer cells. The patients presenting with downregulation of CFHR1 had significantly lower overall survival (OS) and post progression survival (PPS) times. Through analysis of the datasets from Gene Expression Omnibus database, we found that the compound actinomycin D promoted CFHR1 expression, further displaying the cytotoxic effect in the LADC cell line A549. In addition, the expression level of CFHR1 in the cisplatin-resistant LADC cell line CDDP-R (derived from H460) was also ...
The complement inhibitor C4b-binding protein (C4BP) prevents necrotic cells from spilling their pro-inflammatory guts, according to a study on page 1937. Trouw and colleagues now show that C4BP and its binding partner, anticoagulant protein S (PS), cooperate to grab onto necrotic cells and to inhibit the release of cellular DNA.. C4BP short-circuits the complement cascade by binding to the activated complement components C3b and C4b and presenting them to the proteolytic complement inhibitor Factor I for degradation. This inhibitory capacity of C4BP can be coopted by bacterial pathogens, which coat themselves with this protein to avoid complement-mediated destruction by phagocytic cells.. This group recently identified a role for the C4BP-PS complex: it binds to apoptotic cells through the phosphatidylserine-binding domain of PS. This association could prevent the deposition and activation of complement on the surface of the apoptotic cells, allowing the dying cells to be removed without ...
Nineteen years after Gasser et al. [1] reported HUS, an interesting report was published in the Lancet [10]. This report indicated that although C3-predominant activity is initiated in the blood vessels in TMA patients, this is not observed in typical cases of HUS, suggesting that complement activation is involved in aHUS onset [12]. Subsequently, numerous researchers have elucidated further information on the pathology of aHUS. At present, the reported causes of aHUS include, complement regulation abnormalities, cobalamin metabolism disorder, infection with Streptococcus pneumoniae and other microorganisms, drugs, pregnancy, and autoimmune diseases.. The complement system plays an important role as part of the immune systems of living organisms. It is activated via 3 pathways, the classical, alternative, and lectin pathways. As a result of the activation of the hosts alternative and classical pathways, C5b-9, a membrane attack complex, is generated and destroys cells by forming transmembrane ...
Directing selective complement activation towards tumour cells is an attractive strategy to promote their elimination. In the present work, we have generated heteromultimeric immunoconjugates that selectively activate the complement alternative pathway (AP) on tumour cells. We used the C4b-binding protein C-terminal-alpha-/beta-chain scaffold for multimerisation to generate heteromultimeric immunoconjugates displaying (a) a multivalent-positive regulator of the AP, the human factor H-related protein 4 (FHR4) with; (b) a multivalent targeting function directed against erbB2 (HER2); and (c) a monovalent enhanced GFP tracking function. Two distinct VH H targeting two different epitopes against HER2 and competing either with trastuzumab or with pertuzumab-recognising epitopes [VH H(T) or VH H(P)], respectively, were used as HER2 anchoring moieties. Optimised high-FHR4 valence heteromultimeric immunoconjugates [FHR4/VH H(T) or FHR4/VH H(P)] were selected by sequential cell cloning and a selective ...
Background: Cytologic examination of specimens obtained from the respiratory tract is a lung cancer diagnostic procedure with high specificity, but moderate sensitivity. The use of molecular biomarkers may enhance the sensitivity of cytologic examination in the detection of lung cancer. Methods: Complement factor H, a protein secreted by lung cancer cells, was quantified in a series of bronchoalveolar lavage supernatants from lung cancer patients and patients with non-malignant respiratory diseases. Albumin, total protein content, and hemoglobin were also analyzed. Results were validated in independent sets of bronchoalveolar lavage and sputum supernatants. Results: There was a significantly higher concentration of factor H in bronchoalveolar lavage samples from lung cancer patients. The sensitivity and specificity of the factor H test was 82% and 77%, respectively. These results were validated in an independent set of patients with nearly identical results. Furthermore, 70% and 45% of ...
An international team of scientists has identified a protein that is strongly linked to the commonest cause of blindness in developed countries when its levels are raised in the blood. The discovery is a major step forward in the understanding of age-related macular degeneration (AMD), which affects 1.5 million people in the UK alone. The study, carried out by a team from the Universities of Manchester, Cardiff, London, and Nijmegen, and Manchester Foundation NHS Trust was published online on February 7, 2020 in Nature Communications. The open-access article is titled Increased Circulating Levels of Factor H Related Protein 4 Are Strongly Associated with Age-Related Macular Degeneration. The protein, Factor H-Related Protein 4 (FHR4), was found by the team to be present at higher levels in the blood of patients with AMD compared to individuals of a similar age without the disease. The findings were confirmed in 484 patient and 522 control samples from two independent collections across ...
Brocklebank V, Johnson S, Sheerin TP, Marks SD, Gilbert RD, Tyerman K, Kinoshita M, Awan A, Kaur A, Webb N, Hegde S, Finlay E, Fitzpatrick M, Walsh P, Wong EKS, Booth C, Kerecuk L, Salama A, Almond M, Inward C, Goodship TH, Sheerin N, Marchbank KJ, Kavanagh D. Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland. Kidney International 2017, 92(5), Pages 1261-1271 ...
Envisioning A Cure For AMD (by Rick Trevino, OD). I would like to quote from an article by Dr. Rick Travino, who is active in our online community and who hosts one of the most informative web sites about vision research and developments. In his commentary, Envisioning A Cure For AMD, he wrote:. One of the most exciting developments in the field of AMD research has been the discovery of a relatively small number of genes that seems to control a large amount of the risk of developing the disease.. Most of the genes that are known to influence the risk of developing AMD involve various components of the complement cascade. Most prominent among these is complement factor H (CFH); however, variants in genes coding for other components of the complement system have also been discovered and shown to be associated with AMD risk and protection. The complement system is very important in regulating the bodys immune system and directing inflammatory processes. Several lines of evidence suggest that ...
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AbstractObjective: To investigate the association of three single nucleotide polymorphisms (SNPs) in the complement factor H (CFH), KIAA1109, and interleukin-27 (IL-27) genes in patients with anterior uveitis (AU). ...
In a significant development the FDA in the USA approved Soliris (Eculizumab) for use in atypical Hemolytic Uremic Syndrome. To those saying,
The Center for Life at Newcastle upon Tyne, UK, is organizing a conference for patients and their family on atypical Hemolytic Uremic Syndrome on Sat...
Patients. The index family has 2 affected children. In 1987, at the age of 2, patient no. 635, a girl, suffered from urinary tract infection followed by proteinuria and hematuria. Similar symptoms were also found in her older brother (patient no. 638). No other clinical signs were noted, and serology for antinuclear antibody and antineutrophil cytoplasmic autoantibody was negative. The proteinuria was steroid resistant, increased slowly over the years, and developed into nephrotic syndrome. In 2000, renal biopsies of both siblings revealed prominent C3c staining along the GBM and also partly within the mesangium but no relevant immunoglobulin staining. Electron microscopy in one reprocessed paraffin-embedded biopsy showed intense partly ribbon-like, electron-dense deposits within and along the GBM and in the mesangium. The observed images were interpreted as evidence of C3 glomerulopathy in both cases. In the case analyzed by electron microscopy, some features were reminiscent of DDD. Renal ...
This is an interesting follow-up study from Lukiws group on the possible role of microRNAs in AD. Here, the authors identify a novel regulatory pathway (NFκB , miR-146a , complement factor H) potentially involved in the inflammation response in AD brain. Hence, apart from modulating directly BACE1 and Aβ levels (as shown by us and Peter Nelsons group), the microRNA network is emerging as a potential important contributor to AD pathology.. The findings presented in this paper originate from the observation that miR-146a levels are elevated in AD brain. Whether these changes in miR-146a are confirmed in an independent set of patient samples remains to be seen. Indeed, current studies in humans suggest no or minimal overlap in changes in microRNA expression profiles in AD brain. While this does not exclude a role for microRNAs in neurodegeneration-which is clearly supported by studies in animals-it is important to take into consideration the experimental variables which may explain these ...
Information for healthcare professionals for diagnosing and treating Atypical Hemolytic Uremic Syndrome. Soliris is the only therapy approved for the treatment of aHUS.
Emergent plasma exchange at one to one and a half times plasma volume daily is necessary to remove antibody inhibitors of the ADAMTS13 and replete the enzyme and should be started without delay in suspected cases. High dose glucocorticoids have also been shown to help and can be started immediately, for example, prednisone 1mg/kg/day. Rituximab can also be used in certain situations but is mainly used when plasma exchange and steroids do not work.. Typical HUS does not require specific treatment especially in the case of children. Supportive care with fluids is common.. In Atypical HUS, patients with complement H deficiency, eculizumab (a monoclonal protein against complement protein C5) has been shown to be effective for treatment and should be started as soon as a diagnosis is suspected to try to preserve renal function.. ...
aHUS patients raised the question are the predisposing genetic factors of aHUS fully catalogued? as a topic of research which matters to them in their Global Research Agenda.. Those affected by aHUS know well that it is imperfections in components of the Complement System that made them susceptible to the disease when one of many triggering hits over their lives caused a catastrophic onset of aHUS.. They know that there are different imperfections in different aHUS patients, some not yet found. But how many and who is keeping a record of what to look for as an aHUS susceptibility imperfection , or mutation or significant variant.. At University College London, the Department of Structural and Molecular Biology has been collating variants in the Complement System for 15 years and creating a database of information about them. It is known as the Database of Complement Gene Variants and can seen online , click here.. Designed for use by scientist and clinicians the information held is ...
Read about a case report study describing the clinical case of a patient with atypical hemolytic uremic syndrome (aHUS) associated with heart disease.
Kidney biopsy from child with hemolytic uremic syndrome characteristically most likely In hemolytic uremic syndrome there is wide spread formation of hyaline thrombi in the microcirculation, which are composed of dense aggregates of platelets surrounded by fibrin which is referred t
CHESHIRE, CT, Nov. 9, 2013- Soliris® (eculizumab) Inhibits TMA and Improves Renal Function in Pediatric and Adult Patients with atypical Hemolytic Uremic Syndrome (aHUS).
PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... GLA Factor V and factor VIII, combined deficiency of; 227300; MCFD2 Factor V deficiency; 227400; F5 Factor XI deficiency, ... F11 Factor XII deficiency; 234000; F12 Factor XIIIA deficiency; 613225; F13A1 Factor XIIIB deficiency; 613235; F13B Failure of ... LCAT Fletcher factor deficiency; 612423; KLKB1 Focal cortical dysplasia, Taylor balloon cell type; 607341; TSC1 Focal dermal ...
... factor H, factor HR1 or HR3, membrane cofactor protein, factor I, factor B, complement C3, and thrombomodulin). This results in ... The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane ... of complement can result from production of anti-factor H autoantibodies or from genetic mutations in any of several complement ... "Platelet-associated complement factor H in healthy persons and patients with atypical HUS". Blood. 114 (20): 4538-4545. doi: ...
Like complement factor H, CFHR5 is able to bind to complement C3. A mutation in CHFR5 was found in patients with the disease ... "Entrez Gene: CFHR5 complement factor H-related 5". McRae JL, Duthy TG, Griggs KM, et al. (2005). "Human factor H-related ... 2006). "Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... Complement factor H-related protein 5 is a protein that in humans is encoded by the CFHR5 gene. CFHR5 is structurally related ...
Porins are also recognized by TLR2, they bind complement factors (C3b, C4b, factor H, and C4bp (complement factor 4b-binding ... Complement inhibition[edit]. Factor H binding protein (fHbp) that is exhibited in N. meningitidis and some commensal species is ... Close contact with a carrier is the predominant risk factor. Other risk factors include a weakened general or local immune ... Porins are also an important factor for complement inhibition for both pathogenic and commensal species. Porins are important ...
Some HLA alleles have been suspected along with complement phenotypes as being genetic factors. Non-aggressive Berger's disease ... Other blood tests done to aid in the diagnosis include CRP or ESR, complement levels, ANA, and LDH. Protein electrophoresis and ... Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA ... In cases where tonsillitis is the precipitating factor for episodic hematuria, a tonsillectomy has been claimed to reduce the ...
"Cellular adhesion mediated by factor J, a complement inhibitor. Evidence for nucleolin involvement". The Journal of Biological ... Nucleolin is also able to act as a transcriptional coactivator with Chicken Ovalbumin Upstream Promoter Transcription Factor II ...
... is produced when complement factor I cleaves C3b. Complement receptors on white blood cells are able to bind iC3b, so iC3b ... thus preventing amplification of the complement cascade through the alternative pathway. Complement factor I can further cleave ... iC3b is a protein fragment that is part of the complement system, a component of the vertebrate immune system. ... Unlike intact C3b, iC3b cannot associate with factor B, ...
"Onchocerca volvulus microfilariae avoid complement attack by direct binding of factor H". The Journal of Infectious Diseases. ... The complement system is used to enhance the effect of antibodies and phagocytic cells, which engulf and destroy other cells. ...
Complement factors are decreased in rheumatoid arthritis and lupus arthritis. Microscopic analysis of synovial fluid is ... Jay, GD; Britt, DE; Cha, CJ (March 2000). "Lubricin is a product of megakaryocyte stimulating factor gene expression by human ...
The Factor H-related protein 1 (FHR1) has been identified as a novel inhibitor of the complement pathway. FHR1 blocks C5 ... Apparently Factor H and FHR1 control complement activation in a sequential manner. In hemolytic uremic syndrome (HUS), the ... 2009). "Factor H-related protein 1 (FHR-1) inhibits complement C5 convertase activity and terminal complex formation". Blood. ... Factor B then binds to C3b and is cleaved by a plasma serine protease Factor D. The C3bBb complex (= alternative pathway C3 ...
In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and ... In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency ...
Such proteins are referred to as virokines if they resemble cytokines, growth factors, or complement regulators; the term ... The first identified virokine was an epidermal growth factor-like protein found in myxoma viruses. Much of the early work on ... Kotwal, GJ; Moss, B (8 September 1988). "Vaccinia virus encodes a secretory polypeptide structurally related to complement ... which was discovered to secrete proteins that promote proliferation of neighboring cells and block complement immune activity ...
"Evid Based Complement Alternat Med. 7 (1): 11-28. doi:10.1093/ecam/nen023. PMC 2816378. PMID 18955327.. ... Multiple factors such as gender, age, ethnicity, education and social class are also shown to have association with prevalence ... "Who Uses CAM? A Narrative Review of Demographic Characteristics and Health Factors Associated with CAM Use" ...
Overall this protein shows similarity to the complement 1Q factors (C1Q). However, when the 3-dimensional structure of the ... "The crystal structure of a complement-1q family protein suggests an evolutionary link to tumor necrosis factor". Current ... A low level of adiponectin is an independent risk factor for developing: Metabolic syndrome Diabetes mellitus Lower levels of ... Renaldi O, Pramono B, Sinorita H, Purnomo LB, Asdie RH, Asdie AH (January 2009). "Hypoadiponectinemia: a risk factor for ...
Malhotra R, Ward M, Sim RB, Bird MI (1999). "Identification of human complement Factor H as a ligand for L-selectin". Biochem. ... The molecule is composed of multiple domains: one homologous to lectins, one to epidermal growth factor, and two to the ... The embryo secretes human chorionic gonadotropin (hCG), which downregulates anti-adhesion factor, MUC-1, located on the uterine ...
April 2005). "Complement factor H variant increases the risk of age-related macular degeneration". Science. 308 (5720): 419-21 ... Some of the factors that should be considered are the level of efficacy of various genetic tests in the general population, ... 2015-07-02). "Race/Ethnic Differences in the Associations of the Framingham Risk Factors with Carotid IMT and Cardiovascular ... "individual risk factors". The study, Barriers to the Use of Personalized Medicine in Breast Cancer, took two different ...
Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... CFHR4 complement factor H-related 4". Hageman GS, Hancox LS, Taiber AJ, et al. (2007). "Extended Haplotypes in the Complement ... Complement factor H-related protein 4 is a protein that in humans is encoded by the CFHR4 gene. GRCh38: Ensembl release 89: ... 2000). "Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and ...
Complement factor H-related protein 2) at the PDBe-KB. Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: ... Díaz-Guillén MA, Rodríguez de Córdoba S, Heine-Suñer D (1999). "A radiation hybrid map of complement factor H and factor H- ... "Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related ... Complement factor H-related protein 2 is a protein that in humans is encoded by the CFHR2 gene. GRCh38: Ensembl release 89: ...
Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. ... Faktor XII (Hageman Factor) liver A protein that circulates inactively, until activated by collagen, platelets, or exposed ... The combination and activation of this range of complement proteins forms themembrane attack complex, which is able to insert ... A complex of the complement proteins C5b, C6, C7, C8, and multiple units of C9. ...
BCX9930, an oral Factor D inhibitor for the treatment of complement-mediated diseases. FDA has granted Fast Track designation ...
Opsonins include Mfge8, Gas6, Protein S, antibodies and complement factors C1q and C3b. Phagoptosis has multiple functions ... Pathogenic cells such as bacteria can be opsonised by antibodies or complement factors, enabling their phagocytosis and ...
1989). "20 KDa homologous restriction factor of complement resembles T cell activating protein". Biochem. Biophys. Res. Commun ... When complement activation leads to deposition of C5b678 on host cells, CD59 can prevent C9 from polymerizing and forming the ... Ninomiya H, Sims PJ (1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain ... 1992). "Complement regulatory proteins at the feto-maternal interface during human placental development: distribution of CD59 ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 5 is a protein that in humans is encoded by the ARF5 gene. ADP-ribosylation factor 5 (ARF5) is a member ... Shin, O H; Exton J H (August 2001). "Differential binding of arfaptin 2/POR1 to ADP-ribosylation factors and Rac1". Biochem. ... Tsuchiya M, Price SR, Tsai SC, Moss J, Vaughan M (March 1991). "Molecular identification of ADP-ribosylation factor mRNAs and ...
"Entrez Gene: CFHR3 complement factor H-related 3". Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an ... Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... Complement factor H-related protein 3 is a protein that in humans is encoded by the CFHR3 gene. GRCh38: Ensembl release 89: ... 2000). "Complement factor H: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 4 is a protein that in humans is encoded by the ARF4 gene. ADP-ribosylation factor 4 (ARF4) is a member ... "ADP-ribosylation factor 4 small GTPase mediates epidermal growth factor receptor-dependent phospholipase D2 activation". J. ... "ADP-ribosylation factor 4 small GTPase mediates epidermal growth factor receptor-dependent phospholipase D2 activation". J. ...
Complement factor H-related protein 5 (CFHR5) nephropathy is a form of inherited kidney disease which is endemic in Cyprus and ... 26 August 2010). "Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with ... "Recurrence of Complement Factor H-Related Protein 5 Nephropathy in a Renal Transplant". American Journal of Transplantation. 11 ... This is evidence that the disease is caused by a problem with alternative complement pathway regulation, rather than activation ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 3 is a protein that in humans is encoded by the ARF3 gene. ADP-ribosylation factor 3 (ARF3) is a member ... Williger BT, Provost JJ, Ho WT, Milstine J, Exton JH (July 1999). "Arfaptin 1 forms a complex with ADP-ribosylation factor and ... Lee CM, Haun RS, Tsai SC, Moss J, Vaughan M (1992). "Characterization of the human gene encoding ADP-ribosylation factor 1, a ...
Complement 3 (C3) through its interaction with factors B and D (adipsin) generates C3a. In the human body, C3a is rapidly ... Complement peptide C5a, C4a, and C3a receptors". Pharmacol. Rev. 65 (1): 500-43. doi:10.1124/pr.111.005223. PMID 23383423. ...
Overall this protein shows similarity to the complement 1Q factors (C1Q). However, when the 3-dimensional structure of the ... "The crystal structure of a complement-1q family protein suggests an evolutionary link to tumor necrosis factor". Current ... response to tumor necrosis factor. • positive regulation of blood pressure. • response to nutrient levels. • positive ... negative regulation of tumor necrosis factor production. • protein localization to plasma membrane. • negative regulation of ...
This is a type of safeguard to the system, almost like a two-factor authentication method. First, the B cells have to encounter ... Differentiation of mature B cells into plasma cells is dependent upon the transcription factors Blimp-1/PRDM1 and IRF4. ... Immunology: lymphocytic adaptive immune system and complement. Lymphoid. Antigens. *Antigen *Superantigen. *Allergen ...
Neonatal and adult recent thymic emigrants produce IL-8 and express complement receptors CR1 and CR2. JCI insight. 2017-08-17, ... Cell-intrinsic transforming growth factor-beta signaling mediates virus-specific CD8+ T cell deletion and viral persistence in ...
The size of an animal is also a factor in determining diet type (Allen's rule). Since small mammals have a high ratio of heat- ... and herbivores when they are just obtaining nutrients from materials originating from sources that do not seemingly complement ...
"Evid Based Complement Alternat Med. 2012: 1-19. doi:10.1155/2012/837939. PMC 3443820. PMID 22991573.. ... Other factors. There are also reasons why a placebo treatment group may outperform a "no-treatment" group in a test which are ... "J Altern Complement Med. 12 (3): 323-28. doi:10.1089/acm.2006.12.323. PMID 16646733.. ... Social factors. Authors have speculated on the socio-cultural and psychological reasons for the appeal of alternative medicines ...
... between the then-Australian Prime Minister Tony Abbott and Japanese Prime Minister Shinzō Abe had also been cited as a factor ... Complement:. 60[3]. Sensors and processing systems:. AN/BYG-1 combat system[2]. ...
Indeed, automation threatens repetitive jobs but higher-end jobs are still necessary because they complement technology and ... Superpower § Possible factors. Theories and concepts in technology. *Appropriate technology. *Diffusion of innovations ...
... a k-factor is a factor that is k-regular. In particular, a 1-factor is the same thing as a perfect matching. A factor-critical ... complement. The complement graph G. ¯. {\displaystyle {\bar {G}}}. of a simple graph G is another graph on the same vertex set ... factor. A factor of a graph is a spanning subgraph: a subgraph that includes all of the vertices of the graph. The term is ... A graph factorization is a partition of the edges of the graph into factors; a k-factorization is a partition into k-factors. ...
Stated another way, the correlation is the difference between the common language effect size and its complement: r. =. f. −. ( ...
Urbanization and other anthropogenic factors can be implicated in the spread of Lyme disease to humans. In many areas, ... The resistance of a genospecies of Lyme disease spirochetes to the bacteriolytic activities of the alternative complement ... inactivating key immune components such as complement, and hiding in the extracellular matrix, which may interfere with the ... The authors argued that the factors influencing tick density and human risk between sites are still poorly understood, and that ...
"Evid Based Complement Alternat Med. 3 (4): 425-32. doi:10.1093/ecam/nel044. PMC 1697737 . PMID 17173105. Archived from the ... Gaumer G (2006). "Factors associated with patient satisfaction with chiropractic care: survey and review of the literature". J ... J Altern Complement Med. 14 (4): 361-68. doi:10.1089/acm.2007.0766. PMID 18435599.. ... J Altern Complement Med. 13 (5): 491-512. doi:10.1089/acm.2007.7088. PMID 17604553.. ...
"Non-dietary risk factors for gastric dilatation-volvulus in large and giant breed dogs" (PDF). Archived (PDF) from the original ... the trade in dyeing and affixing their fur to unusual proportions began with the need to complement the Victorian and Georgian ...
Outside of DR3-DQ2 with known associations to autoimmune disease, other factors within A1::DQ2 are believed to also contribute ... July 2008). "Autoimmune-associated HLA-B8-DR3 haplotypes in Asian Indians are unique in C4 complement gene copy numbers and HSP ... DR3-DQ2 is either a known or highly suspect factor in most autoimmune diseases that link to the A1::DQ2 haplotype. ... Most important of which is the TNF (tumor necrosis factors) with 3 loci in the region. Starting from B8, immediately followed ...
Full-time employees have an important role in the temple's active approach of spreading Buddhism: they complement monastics who ...
However, killing another male's cubs, upon the takeover, allows the new selected gene complement of the incoming alpha male to ... These studies show that inbreeding depression and ecological factors have an influence on survival.[20] ... breeding or natural environmental factors, the deleterious inherited traits are culled.[6][7][32] ... "Does morality have a biological basis? An empirical test of the factors governing moral sentiments relating to incest" ...
J Altern Complement Med. 4 (3): 289-303. doi:10.1089/acm.1998.4.3-289. PMID 9764768.. ... Temperature requirements and growth rates are crucial factors that identify O. sinensis from other similar fungi.[6] Climate ...
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... 4) β-Scaffold factors. with minor groove contacts. 4.2. *Hyperimmunoglobulin E syndrome ...
It is widely accepted that there is a factor within the tissues of the first branchial arch that is necessary for the ... The location of the canines reflect their dual function as they complement both the premolars and incisors during chewing. ...
When estimating static lung compliance, volume measurements by the spirometer needs to be complemented by pressure transducers ... and so a correction factor is needed to adjust for standard pressure. Online calculators are available to correct for ...
The N terminus interacts with other cellular transcription factors in a ligand-independent manner; and, depending on these ... complement receptors, Fc receptors, B cell receptors and T cell receptors.[12] ...
Factor Xa inhibitors. (with some II inhibition). Heparin group/. glycosaminoglycans/. (bind antithrombin). *Low molecular ... Plasmin, in addition to lysing fibrin clots, also cleaves the complement system component C3, and fibrin degradation products ... Many trauma patients suffer from an overwhelming activation of tissue factor and thus massive hyperfibrinolysis.[6] Also in ... The ELT measures fibrinolysis by clotting the euglobulin fraction (primarily the important fibrinolytic factors fibrinogen, PAI ...
... complement - complement cascade - complementary and alternative therapy - complete blood count (CBC) - computed tomography scan ... granulocyte macrophage-colony stimulating factor (GM-CSF) - granulocyte-colony stimulating factor (G-CSF) - granulocytopenia ... host factors - HPTN - HPV - HRSA - HTLV-I - HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) - HTLV-II - ...
This is complemented by less respectable amusements such as striptease, which, since the 1950s has become a fixture of certain ... These and other factors meant that industries relating to construction, repair, and victualling of naval and merchant ships ...
B cells make antibodies that can bind to pathogens, block pathogen invasion, activate the complement system, and enhance ... Cancer - either by growth factors secreted by the tumor or invasion of bone marrow by the cancer ...
Factor I deficiency (pyogenic infections) Factor H deficiency (haemolytic-uraemic syndrome, membranoproliferative ... Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
The lupus anticoagulant will inhibit all the contact activation pathway factors (factor VIII, factor IX, factor XI and factor ... as well as activation of coagulation and complement pathways.[3] ... Levels of protein C, free and total protein S, factor VIII, ... Risk factors[edit]. Risk factors for developing antiphospholipid syndrome include:[citation needed] ... Further studies for factor V Leiden variant and the prothrombin G20210A mutation, factor VIII levels, MTHFR mutation. ...
Complement system[change , change source]. The complement system is a biochemical cascade of the immune system that helps ... Chemical factors produced during inflammation attract phagocytes, especially neutrophils.[5] Neutrophils then trigger other ... Elements of the complement cascade can be found in many non-mammalian species including plants, birds, fish and some species of ... Starts the complement cascade to identify bacteria, activate cells and clear out dead cells. ...
Other factors that indicate giftedness will also be considered for eligibility.[31] ... a significant complement of teachers, as well as, many full and part-time staff members. The Administrative infrastructure ... depending on a number of factors, such as property values and the personal income of district residents. Act 1 included 10 ...
O'Donnell, J; Laffan MA (August 2001). "The relationship between ABO histo-blood group, factor VIII and von Willebrand factor ... "HIV-1 incorporates ABO histo-blood group antigens that sensitize virions to complement-mediated inactivation". Blood 105 (12 ... 1995). "ABO blood group genotype and plasma von Willebrand factor in normal individuals". Vox Sang 68 (4): 236-40. doi:10.1111/ ... ஆர்எச் காரணி (rhesus factor) மிகவும் முக்கியமானதாகும். எனவே ஆர்எச் குருதி குழு முறைமையும் (Rh Blood group system), இந்த ஏபிஓ ...
... but when all the selection factors are included, the difference is less than 1%. The results are dependent on the intake rather ... but in that year the Elementary Education Act 1870 permitted local governments to complement the existing elementary schools in ...
... genetic factors and environmental factors are thought to be of roughly equal importance.[13] The genetic factors are better ... complemented with laboratory research in compliance with both research protocols and regular monitoring. Thirty of these people ... growth factors, neurotrophic factors, anti-inflammatory drugs, antioxidants, anti-apoptotic drugs, and drugs to improve ... Environmental factors[edit]. Where no family history of the disease is present - around 90% of cases - no cause is known. ...
Complement factor I deficiency is a disorder that affects the immune system. Explore symptoms, inheritance, genetics of this ... or absent complement factor I. The lack (deficiency) of functional complement factor I protein allows uncontrolled activation ... medlineplus.gov/genetics/condition/complement-factor-i-deficiency/ Complement factor I deficiency. ... Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when ...
... Jean Charchaflieh,1 Julie Rushbrook,2 Samrat Worah,2 and Ming Zhang ...
Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the ... C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. UniProt ...
CFHR5 complement factor H related 5 [Homo sapiens] CFHR5 complement factor H related 5 [Homo sapiens]. Gene ID:81494 ... complement factor H-related protein 5. Names. factor H-related protein 5. ... Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important ... Title: Complement factor H-related hybrid protein deregulates complement in dense deposit disease. ...
Schulz, T. F.; Schwaeble, W.; Stanley, K. K.; Weiss, Elisabeth H.; Dierich, Manfred P. (1986): Human complement factor H. ... kDa fragment of human complement control protein factor H using polyclonal and monoclonal antibodies to screen a human liver ...
Factor H-like 1 (FHL-1) is a splice variant of FH that also possesses complement-inhibiting function. Additional five factor H- ... In addition to its canonical role in complement regulation, several other functions of FH have been discovered and a large ... While other fluid-phase and cell membrane-bound regulators of complement have been identified, FH is essential for controlling ... Moreover, numerous pathogenic microbes and some tumor cells have developed the ability to exploit FH for complement evasion. ...
Factor I deficiency in turn leads to low levels of complement component 3 (C3), factor B, factor H and properdin. In blood, due ... Complement factor I (factor I) is a protein of the complement system, first isolated in 1966 in guinea pig serum, that ... Loss of function mutations in the Complement Factor I gene lead to low levels of factor I which results in increased complement ... Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. ...
Complement factor B is a protein that in humans is encoded by the CFB gene. This gene encodes complement factor B, a component ... CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). "The Ba fragment of complement factor B ... Christie DL, Gagnon J (January 1983). "Amino acid sequence of the Bb fragment from complement Factor B. Sequence of the major ... Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the ...
Buy our Recombinant Human Complement factor B protein (denatured). Ab174401 is a protein fragment produced in Escherichia coli ... Recombinant Human Complement factor B protein (denatured). See all Complement factor B proteins and peptides. ... Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb ... a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in ...
Complement factor H gene associations with end-stage kidney disease in African Americans.. Bonomo JA1, Palmer ND2, Hicks PJ3, ... Mutations in the complement factor H gene (CFH) region associate with renal-limited mesangial proliferative forms of ...
Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H. R D Horstmann, H ... Isolated complement components were used to study the regulation of the alternative complement pathway C3 convertase (EC 3.4. ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ...
Staphylococcus aureusClumping Factor A Binds to Complement Regulator Factor I and Increases Factor I Cleavage of C3b ... Myeloperoxidase influences the complement regulatory activity of complement factor H. Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li ... Factor H and Factor H-Related Protein 1 Bind to Human Neutrophils via Complement Receptor 3, Mediate Attachment to Candida ... Acquisition of Complement Inhibitor Serine Protease Factor I and Its Cofactors C4b-Binding Protein and Factor H by Prevotella ...
Complement factor H-related hybrid protein deregulates complement in dense deposit disease. Qian Chen,1 Michael Wiesener,2 ... Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... Then, factor B (2.5 μg/ml, CompTech), factor D (0.5 μg/ml, CompTech), and factor P (2 μg/ml, CompTech), together with the CFHR2 ... Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related ...
Browse our Complement Factor H Lysate catalog backed by our Guarantee+. ... Complement Factor H Lysates available through Novus Biologicals. ... Complement Factor H Lysates. We offer Complement Factor H ... factor H lysate, factor H-like 1 lysate, FH lysate, FHL1 lysate, H factor 1 (complement) lysate, H factor 1 lysate, H factor 2 ... Alternate Names for Complement Factor H Lysates. Complement Factor H lysate, CFH lysate, adrenomedullin binding protein lysate ...
Browse our Complement Factor H Antibodies all backed by our Guarantee+. ... Complement Factor H Antibodies available through Novus Biologicals. ... anti-H factor 1 (complement) antibody, anti-H factor 1 antibody, anti-H factor 2 (complement) antibody, anti-HF antibody, anti- ... Complement Factor H Antibodies. We offer Complement Factor H Antibodies for use in common research applications: ELISA, Flow ...
J:277202 Song D, et al., Complement Factor H Mutation W1206R Causes Retinal Thrombosis and Ischemic Retinopathy in Mice. Am J ...
A growing number of pathogenic microorganisms have acquired the ability to bind the complement inhibitor factor H (FH) from ... A growing number of pathogenic microorganisms have acquired the ability to bind the complement inhibitor factor H from body ... Initial studies assumed that these proteins are complement inhibitors similar to factor H. However, recent evidence suggest ... In addition to factor H, binding of factor H-related proteins was also demonstrated for several microbes. ...
Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ...
CRP-Induced Expression of Complement- Inhibitory Factors Protects HSVECs From Complement-Mediated Injury. To assess whether the ... 13 The effect of CRP on the expression of complement-inhibitory factors is unknown. Because complement-inhibitory proteins ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ...
The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ... Complement Factor 9 Deficiency in Serum of Human Neonates J Infect Dis. 1992 Jul;166(1):53-7. doi: 10.1093/infdis/166.1.53. ... The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ...
... factor VIII deficiency (ortholog); FOUND IN extracellular space (inferred); secretory granule (inferred) ... INVOLVED IN complement activation, alternative pathway (inferred); ASSOCIATED WITH autistic disorder (ortholog); Experimental ... Homo sapiens (human) : CFP (complement factor properdin) HGNC Alliance Mus musculus (house mouse) : Cfp (complement factor ... Sus scrofa (pig) : CFP (complement factor properdin) Chlorocebus sabaeus (African green monkey) : CFP (complement factor ...
... products and learn more about anti-Complement Factor D/Adipsin, Polyclonal, Novus Biologicals 0.1mg; 0.1mg; Unlabeled. ... Adipsin, ADNcomplement factor D, C3 convertase activator, complement factor D (adipsin), complement factor D preproprotein, D ... component of complement (adipsin), DF, EC 3.4.21, EC 3.4.21.46, PFD, Properdin factor DADIPSIN. ...
Since there is complement activation in all areas of tissue injury, and both C3a and C5a activate MSC, it was asked whether ... The signaling cascade responsible for the production of angiogenic factors by C3a or C5a could be defined as activation of the ... C3a caused significant up-regulation of various angiogenic factors, including VEGF, CXCL8/IL-8 and IL-6. In contrast there was ... Although C5a also caused moderate up-regulation of angiogenic factors, the effect was borderline significant. Furthermore the ...
Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ... Among 116,204 single-nucleotide polymorphisms genotyped, an intronic and common variant in the complement factor H gene (CFH) ...
Both age and smoking, two important risk factors for AMD, influence plasma levels of complement factor H (28). CFH sequences ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ...
Structures of native and complexed complement factor D: implications of the atypical His57 conformation and self-inhibitory ... R-Factor (R-Work). R-Factor (R-Free). R-Factor (R-Free Error). Percent Reflections (Observed). ... R-Factor (All). R-Factor (Observed). R-Work. R-Free. R-Free Selection Details. ...
Complement Factor H Antibody Biotin conjugate (L20/3), GAU 020-03B-01, from Invitrogen™. Species Reactivity: Human; ... Factor H is a regulatory factor of the alternative pathway of complement activation. Factor H is a glycoprotein consisting of a ... GAU 020-03B-01 detects Complement factor H (ß1H-globulin) in human samples. GAU 020-03B-01 has been successfully used in ELISA ... GAU 020-03B-01 immunogen is human Factor H. NOTE: Concentration is lot-dependent and can vary from 0.85-1.15 mg/mL ...
Mouse podocyte complement factor h: the functional analog to human complement receptor 1. Alexander, J.J., Wang, Y., Chang, A ... Complement factor H (Cfh) is a key plasma protein in humans and animals that serves to limit alternative pathway complement ... Complement factor h limits immune complex deposition and prevents inflammation and scarring in glomeruli of mice with chronic ... Distinct and separable roles of the complement system in factor h-deficient bone marrow chimeric mice with immune complex ...
If you are a society or association member and require assistance with obtaining online access instructions please contact our Journal Customer Services team ...
Complement factor C5a mediates renal ischemia-reperfusion injury independent from neutrophils. de Vries, B., Kohl, J., Leclercq ... However, the contribution of complement factor C5a to I/R injury, in particular in the kidney, remains to be established. In ... The complement system has been shown to mediate renal ischemia-reperfusion (I/R) injury. ...
  • Complement factor I and several related proteins protect healthy cells by preventing activation of the complement system when it is not needed. (medlineplus.gov)
  • Additional five factor H-related (FHR) proteins belong to the FH family and have been recognized as competitors of FH for binding to various ligands, thus modulating complement regulation. (frontiersin.org)
  • Initial studies assumed that these proteins are complement inhibitors similar to FH. (frontiersin.org)
  • However, recent evidence suggests that FHR proteins may rather enhance complement activation both directly and also by competing with the inhibitor FH for binding to certain ligands and surfaces. (frontiersin.org)
  • This mini review focuses on the role of the main alternative pathway regulator FH in host-pathogen interactions, as well as on the emerging role of the FHR proteins as enhancers of complement activation. (frontiersin.org)
  • The complement system, a major humoral arm of innate immunity, includes ~40 plasma and cell membrane-anchored proteins that act in a cascade-like manner to opsonize microbes and facilitate their phagocytosis, activate cellular responses, initiate inflammation, or directly lyse certain microbes by punching holes into them ( 1 ). (frontiersin.org)
  • (B) The five human FH-related (FHR) proteins retained domains homologous to complement control protein domains 6-9 and 18-20 of FH (showed by vertical alignment). (frontiersin.org)
  • Because complement is a powerful system to facilitate destruction of microbes or other target cells, host cells and tissues are protected by various combinations of fluid phase and membrane complement regulatory proteins that fine tune and/or block the activation steps of the complement cascade, restrict activation in both time and space, and prevent the potential deleterious effects of full-blown, excessive activation (Figure 1 A) ( 3 ). (frontiersin.org)
  • Methods and Results- Human coronary artery or human saphenous vein ECs were incubated with CRP (0 to 100 μg/mL, 0 to 72 hours), and the expression of the complement-inhibitory proteins decay-accelerating factor (DAF), membrane cofactor protein (CD46), and CD59 were measured by flow cytometry. (ahajournals.org)
  • The increased expression of complement-inhibitory proteins was functionally effective, resulting in significant reduction of complement-mediated lysis of antibody-coated human saphenous vein ECs. (ahajournals.org)
  • The complement system is a complex cascade of enzymes and regulatory proteins that normally participate in host defenses against microorganisms via opsonization, chemoattraction of leukocytes, cell lysis, and cell activation. (ahajournals.org)
  • Among these regulatory proteins is decay-accelerating factor (DAF, CD55), membrane cofactor protein (MCP, CD46), which binds to and facilitates the degradation of C3b and C4b, and CD59, which inhibits C5b-9. (ahajournals.org)
  • Because complement-inhibitory proteins protect the endothelium from complement-mediated injury and CRP is proatherogenic, in part because of its ability to activate complement, we hypothesized that the detrimental effect of CRP may involve the downregulation of these protective proteins. (ahajournals.org)
  • In analogy to the newly described neuroimmune regulatory proteins also known as "don't eat me" signals (CD200, CD47, CD22, fractalkine, semaphorins), we herein identify the key role of complement regulator factor H (fH) in controlling neuroinflammation initiated in an acute mouse model of Ab-dependent experimental autoimmune encephalomyelitis. (jimmunol.org)
  • Interactions of factor H with various endogenous ligands, such as pentraxins, extracellular matrix proteins and DNA are important in limiting local complement-mediated inflammation. (mdpi.com)
  • VEGF regulates local inhibitory complement proteins in the eye and kidney. (lsbio.com)
  • Unexpectedly, levels of factor H in bronchoalveolar samples correlate with levels of albumin, suggesting that the cause of factor H accumulation in bronchial fluid is not its direct secretion from tumor cells but the exudation of plasma proteins to the bronchial lumen. (aacrjournals.org)
  • Common polymorphisms in complement alternative pathway (AP) proteins C3 (C3R102G), factor B (fBR32Q), and factor H (fHV62I) are associated with age-related macular degeneration (AMD) and other pathologies. (csic.es)
  • The complement system is a component of innate immunity produced locally in the CNS, since size restrictions from the blood brain barrier prevent complement proteins from easily passing through from the rest of the body. (uiowa.edu)
  • fH is a member of the genetically and structurally related regulators of complement activation family of proteins. (asm.org)
  • Some microbial pathogens utilize human complement regulatory proteins, such as factor H (FH) and factor H-like protein 1 (FHL-1), for immune evasion. (asm.org)
  • The common pathway for the development of pathogen immunity is to evade complement attack and opsonophagocytosis, which is often influenced or dictated by a pathogen's ability to bind complement regulatory proteins ( 1 , 7 , 8 , 12 , 13 , 15 , 17 , 28 ). (asm.org)
  • 26 ) have identified FbaA, which is expressed by a serotype M1 GAS isolate, 90-226, as the protein that mediates the binding of both human complement regulatory proteins FH and factor H-like protein (FHL-1) ( 25 ). (asm.org)
  • We used fluorescent microscopy, monospecific antibodies against complement components, fluorescent secondary antibodies, and the analysis of .150 images to quantify the attachment of HUVEC-released complement proteins to ULVWF strings secreted by, and anchored to, the HUVECs (under conditions of ADAMTS-13 inhibition). (rice.edu)
  • Complement acts as a danger‐sensing system in the innate immune system, and its activation initiates a strong inflammatory response and cleavage of the proteins C3 and C5 by proteolytic enzymes, the convertases. (embopress.org)
  • Activation of the proteolytic complement cascade triggers cleavage of the homologous 185-200 kDa proteins C3, C4, and C5 ( Supplementary Figure S1 ). (embopress.org)
  • Terminal complement proteins C5b-9 release basic fibroblast growth factor and platelet-derived growth factor from endothelial cells. (rupress.org)
  • Endothelial deposits of C5b-9 proteins, the membrane attack complex of complement (MAC), have been found in a variety of pathological tissues in which cell proliferation is an early characteristic abnormality, including atherosclerosis. (rupress.org)
  • We have explored a possible bridging role for terminal complement C5b-9 proteins in eliciting focal signals for cell proliferation by releasing growth factors from endothelial cells. (rupress.org)
  • Complement factor I deficiency is caused by mutations in the CFI gene. (medlineplus.gov)
  • This gene provides instructions for making a protein called complement factor I. This protein helps regulate a part of the body's immune response known as the complement system . (medlineplus.gov)
  • Mutations in the CFI gene that cause complement factor I deficiency result in abnormal, nonfunctional, or absent complement factor I. The lack (deficiency) of functional complement factor I protein allows uncontrolled activation of the complement system. (medlineplus.gov)
  • This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. (nih.gov)
  • Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. (nih.gov)
  • The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. (nih.gov)
  • Rare Variants in the Complement Factor H-Related Protein 5 Gene Contribute to Genetic Susceptibility to IgA Nephropathy. (nih.gov)
  • Atypical hemolytic uremic syndrome and genetic aberrations in the complement factor H-related 5 gene. (nih.gov)
  • Complement factor H gene associations with end-stage kidney disease in African Americans. (nih.gov)
  • Mutations in the complement factor H gene (CFH) region associate with renal-limited mesangial proliferative forms of glomerulonephritis including IgA nephropathy (IgAN), dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). (nih.gov)
  • Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. (wikipedia.org)
  • The gene for Factor I in humans is located on chromosome 4. (wikipedia.org)
  • Loss of function mutations in the Complement Factor I gene lead to low levels of factor I which results in increased complement activity. (wikipedia.org)
  • Complement factor B is a protein that in humans is encoded by the CFB gene. (wikipedia.org)
  • This gene encodes complement factor B, a component of the alternative pathway of complement activation. (wikipedia.org)
  • The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2. (wikipedia.org)
  • Entrez Gene: CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). (wikipedia.org)
  • Molecular cloning and characterization of the gene coding for human complement protein factor B". Proceedings of the National Academy of Sciences of the United States of America. (wikipedia.org)
  • Complement factor 5 is a quantitative trait gene that modifies liver fibrogenesis in mice and humans," Nature Genetics , vol. 37, no. 8, pp. 835-843, 2005. (hindawi.com)
  • Wild-type C57 mice and factor B gene knockout C57 (FBKO) mice were sued. (arvojournals.org)
  • CFH / Complement Factor H is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. (lsbio.com)
  • Four complement factor H gene polymorphisms in association with AMD: A meta-analysis. (semanticscholar.org)
  • Alternatively spliced transcripts of the human complement C2 gene. (semanticscholar.org)
  • This gene encodes a serine proteinase that is essential for regulating the complement cascade. (mybiosource.com)
  • Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. (mybiosource.com)
  • Mutations in this gene underlie complement factor D deficiency, which is associated with recurrent bacterial meningitis infections in human patients. (nih.gov)
  • Complement can be activated by three major pathways, the classical, the lectin, and the alternative pathway. (frontiersin.org)
  • The recognition molecules of the pathways initiate activation by interacting with enzymatically active components that propagate the cascade and generate active complement fragments and complexes that mediate the biological effects of the system (Figure 1 A) ( 2 ). (frontiersin.org)
  • Complement pathways and the human factor H (FH) protein family. (frontiersin.org)
  • Schematic overview of the major complement activation and regulation pathways. (frontiersin.org)
  • You need to have more background on the various complement factors, their function, the 3 different pathways of activation etc) NCIPH is a liver disorder of vascular origin defined by a portal venous pressure exceeding 5mm Hg between portal vein and inferior vena cava (sanyal 2008), characterized by occlusion of the 3rd /4th order branches of the hepatic portal vein ( madhu 2008). (ukessays.com)
  • Complement Factor I (CFI) is a serum protease capable of inhibiting all complement pathways. (arvojournals.org)
  • Background: Vascular endothelial cells (ECs) express and release protein components of the complement pathways, as well as secreting and anchoring ultra-large von Willebrand factor (ULVWF) multimers in long string-like structures that initiate platelet adhesion during hemostasis and thrombosis. (rice.edu)
  • and C4 of the classical and lectin (but not alternative) complement pathways. (rice.edu)
  • In all three complement pathways, the central molecule is C3, which, upon activation cleavage, forms the major opsonin C3b - the key component of complement. (mysciencework.com)
  • Some bacteria are able to resist phagocytosis and interfere with the body's complement pathways. (ccbcmd.edu)
  • Complement factor I deficiency is a disorder that affects the immune system. (medlineplus.gov)
  • Some people with complement factor I deficiency have a kidney disorder called glomerulonephritis with isolated C3 deposits. (medlineplus.gov)
  • Complement factor I deficiency can also be associated with autoimmune disorders such as rheumatoid arthritis or systemic lupus erythematosus (SLE). (medlineplus.gov)
  • Baracho GV, Nudelman V, Isaac L. Molecular characterization of homozygous hereditary factor I deficiency. (medlineplus.gov)
  • Grumach AS, Leitão MF, Arruk VG, Kirschfink M, Condino-Neto A. Recurrent infections in partial complement factor I deficiency: evaluation of three generations of a Brazilian family. (medlineplus.gov)
  • Ponce-Castro IM, González-Rubio C, Delgado-Cerviño EM, Abarrategui-Garrido C, Fontán G, Sánchez-Corral P, López-Trascasa M. Molecular characterization of Complement Factor I deficiency in two Spanish families. (medlineplus.gov)
  • The molecular basis of hereditary complement factor I deficiency. (medlineplus.gov)
  • Factor I deficiency in turn leads to low levels of complement component 3 (C3), factor B, factor H and properdin. (wikipedia.org)
  • We present a case suggesting that complement factor H deficiency, a known hereditary risk factor for TMA, may also favor development of AAG. (nih.gov)
  • We discuss the potential implications of factor H deficiency in the pathophysiology of renal allograft microvascular injury, leukocyte infiltration and formation of intraglomerular platelet thrombi. (nih.gov)
  • Homozygous complement factor deficiency and primary antiphospholipid syndrome: a clinical and serological study. (bmj.com)
  • Thus, deficiency of CFHR3 and CFHR1 results in a loss of complement control but enhances local regulation by factor H. Our findings allude to a critical balance between the complement regulators CFHR3, CFHR1 and factor H and further emphasize the central role of complement regulation in AMD pathology. (uni-regensburg.de)
  • 2. a preparation of factor VIII administered intravenously for the prevention or treatment of hemorrhage in patients with hemophilia A and the treatment of von Willebrand disease , hypofibrinogenemia , and coagulation factor XIII deficiency. (thefreedictionary.com)
  • Deficiency of factor H, or a functional defect in its N terminus, often leads to membranoproliferative glomerulonephritis and complement depletion, owing to continuous overconsumption of C3. (mysciencework.com)
  • Properdin is a positive regulator of complement activation. (frontiersin.org)
  • While other fluid-phase and cell membrane-bound regulators of complement have been identified, FH is essential for controlling complement activation on various cellular and non-cellular surfaces, including basement membranes. (frontiersin.org)
  • Impaired regulatory as well as ligand and cell recognition functions of factor H, caused by mutations or autoantibodies, are associated with the kidney diseases: atypical hemolytic uremic syndrome and dense deposit disease and the eye disorder: age-related macular degeneration. (mdpi.com)
  • The complement system has been implicated in the pathogenesis of Age-related Macular Degeneration (AMD). (arvojournals.org)
  • Plasma complement components and activation fragments: associations with age-related macular degeneration genotypes and phenotypes. (umassmed.edu)
  • Age-related macular degeneration (AMD) is the leading cause of permanent vision loss among the elderly in many industrialized countries, and the complement system plays an important role in the pathogenesis of AMD. (molvis.org)
  • A frequent deletion of complement factor H (CFH)-related genes CFHR3 and CFHR1 (ΔCFHR3/CFHR1) is considered to have a protective effect against age-related macular degeneration (AMD), although the underlying mechanism remains elusive. (uni-regensburg.de)
  • In addition, recently, a polymorphism in the middle part of factor H (Y402H) has been shown to be the major risk factor for the most common cause of blindness in the industrialized world: age-related macular degeneration. (mysciencework.com)
  • Is complement factor H a shared risk factor for age-related macular degeneration and cardiovascular disease? (ucl.ac.uk)
  • Increased activation of the alternative complement pathway in vitreous was controlled by disease stage and genetic variation in the complement pathway, supporting a role for complement activation in macular degeneration disease pathogenesis. (nih.gov)
  • CFD regulates activation of the alternative complement pathway, which is implicated in age related macular degeneration pathogenesis. (nih.gov)
  • Polymorphisms in factor H (FH), a major regulator of complement activation, and the accumulation of high zinc concentrations in the outer retina are both associated with age-related macular degeneration. (qub.ac.uk)
  • The relevance of metal-induced FH oligomer formation to complement regulation and age-related macular degeneration is discussed. (qub.ac.uk)
  • Each Complement Factor H Antibody is fully covered by our Guarantee+, to give you complete peace of mind and the support when you need it. (novusbio.com)
  • Choose from our Complement Factor H polyclonal antibodies and browse our Complement Factor H monoclonal antibody catalog. (novusbio.com)
  • Spitzer RE, Stitzel AE, Tsokos G. On the origin of C3 nephritic factor (antibody to the alternative pathway C3 convertase): evidence for the Adam and Eve concept of autoantibody production. (harvard.edu)
  • Immunoprecipitation analysis of human plasma using anti-Complement factor H antibody. (acris-antibodies.com)
  • AM20110PU-N Factor H antibody staining of Formalin-Fixed, Paraffin-Embedded Human Liver followed by biotinylated secondary antibody, alkaline phosphatase-streptavidin and chromogen. (acris-antibodies.com)
  • Complement Factor B antibody LS-C128908 is an unconjugated goat polyclonal antibody to human Complement Factor B (CFB). (lsbio.com)
  • Strains of group A streptococci lacking M protein are efficiently opsonized by the alternative pathway of complement, but in the absence of type-specific antibody neither the alternative nor the classical pathway is activated by strains expressing M protein ( 3 , 29 ). (asm.org)
  • Anti-Factor I antibody IHC of human colon, vessels. (mybiosource.com)
  • Whereas much attention has been focused on the properties and activities of the TLRs in this process ( 13 ), many other innate immune molecules expressed by glia and neurons have been described (e.g., complement, lectins, scavenger receptors) ( 14 ). (jimmunol.org)
  • Complement is an essential part of innate immunity as it participates in host defense against infections, disposal of cellular debris and apoptotic cells, inflammatory processes and modulation of adaptive immune responses. (mdpi.com)
  • The complement system is a fundamental element of the innate immune system as well as the adaptive immune responses. (ukessays.com)
  • While a role for the complement system on innate as well as adaptive immunity has been documented, the implication of complement activation on the onset of the anti-FVIII immune response is unknown. (haematologica.org)
  • This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. (lsbio.com)
  • The complement system, an important component of innate immunity, is the most widely accepted pathogenic pathway of the immune system implicated in AMD. (molvis.org)
  • have found one such mechanism-cross-talk between the alternative complement pathway and natural killer (NK) cells and innate lymphoid cells (ILCs). (sciencemag.org)
  • Despite its important role in innate immunity, very little is known about complement production, regulation, and function in the CNS of healthy or diseased individuals. (uiowa.edu)
  • Neither M- nor M+ streptococci directly affected the formation or dissociation of the surface-bound C3b,Bb or the inactivation of surface-bound C3b by factor I. However, the activity of the serum control protein of the alternative complement pathway, factor H, in controlling streptococcus-bound C3b and C3b,Bb was 6-8 times stronger on M+ organisms than on M- organisms. (pnas.org)
  • The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 micrograms/ml in each of their 14 neonates. (nih.gov)
  • Complement factor I (factor I) is a protein of the complement system, first isolated in 1966 in guinea pig serum, that regulates complement activation by cleaving cell-bound or fluid phase C3b and C4b. (wikipedia.org)
  • 1. The activation of the protease of blood serum or plasma by streptococcal fibrinolysin requires the presence of both a heat-stable and a heat-labile factor. (jimmunol.org)
  • 3. Useful sources of the heat-labile factor include guinea-pig serum, rabbit plasma, duck serum, and chicken serum. (jimmunol.org)
  • Complement-mediated borreliacidal effects were observed with particular combinations of host serum and Borrelia genospecies. (asm.org)
  • The present study sought to determine whether the generation of chemotactic factor by endotoxin in serum was dependent upon complement system activation. (eurekamag.com)
  • Preheating serum, incubating at 0 degrees C, or incubating in the presence of EDTA, all prevented chemotactic factor generation as well as complement fixation by endotoxin. (eurekamag.com)
  • These experiments demonstrate that generation of chemotactic factor by endotoxin in serum is dependent upon C' system activation involving at least C'5. (eurekamag.com)
  • Group A streptococci possess a wide variety of virulence factors, including M protein, hyaluronic acid capsule, serum opacity factor, C5a peptidase, and extracellular enzymes and toxins ( 8 ). (asm.org)
  • We will now look at the ability of bacteria to resist phagocytic destruction and complement serum lysis. (ccbcmd.edu)
  • FBKO mice were more vulnerable to fungal infection and showed a longer disease course than wild-type C57 mice, suggesting that factor B play an important role in the pathogenesis and prognosis of FK. (arvojournals.org)
  • Thus, pathogenesis in NCIPH patients could be either defective Vwf multimeric processing by ADAMTS13, excess expression of multimeric vWF which overwhelms the cleavage capacity of ADAMTS13 or any other factor which interferes with this system. (ukessays.com)
  • Post-infectious PGNMID and PIGN might share common clinical presentations and pathogenesis related to the complement pathway. (eurekamag.com)
  • Inappropriate activation of the complement cascade has been implicated in the pathogenesis of AD. (qub.ac.uk)
  • In this section on Bacterial Pathogenesis we are looking at virulence factors that promote bacterial colonization of the host . (ccbcmd.edu)
  • To date, the significance and functional implications of tyrosine nitration of complement factor H (CFH), a key complement regulator in the eye has not been explored, and is examined in this study in the context of AMD pathogenesis. (monash.edu)
  • To prevent host cell damage, nucleated cells have developed membrane-bound regulators of complement activation. (ahajournals.org)
  • Several studies reported links between genetic variations of FH and disease, thus confirming the important role of FH in the regulation of complement activation. (frontiersin.org)
  • The complement Factor H protein is secreted into the bloodstream and acts in the regulation of complement activation. (acris-antibodies.com)
  • Background- Because complement-mediated vascular injury participates in atherosclerosis and C-reactive protein (CRP) can activate the complement cascade, we sought to determine whether CRP affects the expression of the protective complement-inhibitory factors on the cell surface of endothelial cells (ECs). (ahajournals.org)
  • 11 DAF prevents the formation and accelerates the decay of the C3 and C5 convertases that act early within the complement cascade, 11 functioning to maintain vascular integrity as a key protector against complement-mediated cell lysis. (ahajournals.org)
  • The signaling cascade responsible for the production of angiogenic factors by C3a or C5a could be defined as activation of the rho cascade which was necessary for nuclear translocation of NF κ B p65 and of phospho-ERK1/2. (scirp.org)
  • In summary, this study shows that enhancive miR-146a can upregulate the inflammatory factor IL-1β in chronic TLE by downregulating CFH, and that upregulation of IL-1β plays an important feedback-regulating role in the expression of miR-146a and CFH, forming a miR-146a-CFH-IL-1β loop circuit that initiates a cascade of inflammation and then leads to the perpetuate inflammation in TLE. (biologists.org)
  • Complement factor H (CFH) is a regulator of the cascade, but studies on plasma CFH levels in AD have provided mixed results. (qub.ac.uk)
  • In conclusion, on the basis of these and previous results, it appears that DAF plays a central role in preventing the amplification of the complement cascade on host cell surfaces. (rupress.org)
  • C3b is also essential for propagation of the complement cascade to the stage of the lytic terminal complement complexes. (mysciencework.com)
  • Evidence for non-traditional activation of complement factor C3 during murine liver regeneration," Molecular Immunology , vol. 45, no. 11, pp. 3125-3132, 2008. (hindawi.com)
  • C3a and C3b activation products of the third component of complement (C3) are critical for normal liver recovery after toxic injury," Journal of Immunology , vol. 173, no. 2, pp. 747-754, 2004. (hindawi.com)
  • Human complement factor H. isolation of cDNA clones and partial cDNA sequence of the 38-kDa tryptic fragment containing the binding site for C3b. (uni-muenchen.de)
  • We isolated cDNA clones coding for the functionally important tryptic N-terminal38- kDa fragment of human complement control protein factor H using polyclonal and monoclonal antibodies to screen a human liver cDNA library cloned in a bacterial expression vector, PEX-1. (uni-muenchen.de)
  • Complement factor B Bb fragment. (abcam.com)
  • Immunochemical studies of the alkaline-denatured C3 suggested that factor H interacts with surfaces of C3 that are situated within the C3c fragment and that are defined by C3(SN) antigens, while factor I predominantly interacts with C3(SN) antigens associated with the C3d fragment and with C3(D) antigens hidden in native C3. (diva-portal.org)
  • Anti factor D Fab fragment inhibits FD proteolytic function by interfering with macromolecular substrate access rather than by inhibiting FD catalysis. (nih.gov)
  • Factor H is released or modified following this cleavage. (acris-antibodies.com)
  • In vitro , complement C3 and its cleavage product C3b enhanced FVIII endocytosis by dendritic cells and presentation to a FVIII-specific CD4 + T-cell hybridoma. (haematologica.org)
  • fH regulates complement activation by acting as a cofactor for factor I-dependent cleavage of C3b ( 27 ) and by disrupting the alternative pathway C3 convertase ( 35 , 37 ). (asm.org)
  • There was loss of cofactor activity for Factor I mediated cleavage of C3b with nitrated CFH compared to non-nitrated CFH. (monash.edu)
  • This protease catalyzes the cleavage of factor B, the rate-limiting step of the alternative pathway of complement activation. (nih.gov)
  • Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. (rcsb.org)
  • Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. (abcam.com)
  • Isolated complement components were used to study the regulation of the alternative complement pathway C3 convertase (EC 3.4.21.47), also called C3b,Bb, on M protein-carrying (M+) and M protein-lacking (M-) streptococci. (pnas.org)
  • Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 convertase of the alternate pathway. (uniprot.org)
  • Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. (wikipedia.org)
  • The classical pathway of complement may be activated by CRP bound to enzymatically degraded low-density lipoprotein. (ahajournals.org)
  • Factor H is a regulatory factor of the alternative pathway of complement activation. (fishersci.com)
  • Although rho was only transiently activated, inhibition of the rho or "downstream of it" of the NF κ B pathway, prevented C3a-and C5a-induced up-regulation of angiogenic factors. (scirp.org)
  • Increased circulating fH and fB concentrations in subjects with altered glucose tolerance could reflect increased SVC-induced activation of the alternative pathway of complement in omental adipose tissue linked to insulin resistance and metabolic disturbances. (diabetesjournals.org)
  • Factor H is a major soluble regulator of the alternative complement pathway, but it can also bind to host cells and tissues, protecting them from complement attack. (mdpi.com)
  • Complement Factor D, also known as adipsin, is a serine protease that catalyzes the initial proteolytic step in the alternative pathway of complement. (rndsystems.com)
  • Complement and the alternative pathway play an important role in LPS/D-GalN-induced fulminant hepatic failure," PloS ONE , vol. 6, no. 11, Article ID e26838, 2011. (hindawi.com)
  • Inhibition of complement factor B, a key regulator of the alternative pathway, is implicated as a potential therapeutic intervention for AMD. (molvis.org)
  • The results suggest that inhibition of liver factor B mRNA by factor B ASOs would reduce systemic alternative complement pathway activation and has potential to be used as a novel therapy for AMD. (molvis.org)
  • CFD, also known as adipsin, is involved in the alternative complement pathway of the complement system where it cleaves factor B. This protein is a member of the trypsin family of peptidases. (acris-antibodies.com)
  • It is a component of the alternative complement pathway best known for its role in humoral suppression of infectious agents. (acris-antibodies.com)
  • CFB / Complement Factor B is complement factor B, a component of the alternative pathway of complement activation. (lsbio.com)
  • A heterozygous complement factor H mutation was detected by comprehensive genetic testing of alternative pathway regulatory genes, which might lead to persistent infection-triggered alternative pathway activation and account for severe glomerulonephritis. (eurekamag.com)
  • Complement factor H (CFH) is an abundant plasma glycoprotein that regulates the function of the alternative complement pathway in fluid phase and on cellular surfaces (PMID: 2963625). (ptglab.com)
  • Factor H binds to C3b, accelerates the decay of the alternative pathway C3-convertase (C3bBb) and acts as a cofactor for the factor I-mediated proteolytic inactivation of C3b (PMID: 15163532). (ptglab.com)
  • They report that complement factor P (CFP), a positive regulator of the alternative complement pathway, binds NKp46, which is expressed on subsets of NK cells and ILC1 and ILC3. (sciencemag.org)
  • These data suggest that ILCs and the alternative complement pathway cooperate to fight off bacterial infection. (sciencemag.org)
  • The complement pathway contributes to inflammatory cell recruitment, cell lysis, and opsonization, and thus requires regulation to avoid inappropriate activation. (uiowa.edu)
  • a regulator of the alternative pathway of complement activation. (uiowa.edu)
  • Complement factor H (CFH) is a soluble complement regulatory protein essential for the down-regulation of the alternative pathway on interaction with specific markers on the host cell surface. (biochemj.org)
  • The alternative complement pathway (AP) is an important nonantibody- requiring host defense system. (rice.edu)
  • The alternative pathway (AP) C3 convertase C3bBb is formed when factor B (fB) combines with C3b, after which fB is activated by factor D (fD) ( Figure 1A ). (embopress.org)
  • The DAF inhibitory effect on EAC14 or EAC43 was not overcome by supplying an excess of C2 or factor B, but the alternative pathway C3 convertase could be assembled in the presence of Ni++, or nonphysiological concentrations of Mg++, which enhances the binding affinity of factor B for C3b. (rupress.org)
  • factor B a complement component that participates in the alternative complement pathway. (thefreedictionary.com)
  • The factor I heavy chain has four domains: an FI membrane attack complex (FIMAC) domain, CD5 domain, and low density lipoprotein receptor 1 and 2 (LDLr1 and LDLr2) domains. (wikipedia.org)
  • the heavy chain plays an inhibitory role in maintaining the enzyme inactive until it meets the complex formed by the substrate (either C3b or C4b) and a cofactor protein (Factor H, C4b-binding protein, complement receptor 1, and membrane cofactor protein). (wikipedia.org)
  • Kinetic analysis of the interactions of complement receptor 2 (CR2, CD21) with its ligands C3d, iC3b, and the EBV glycoprotein gp350/220. (semanticscholar.org)
  • We have begun to address whether these responses reflect the same sets of conformational changes in the receptor using constitutively active mutants of the human complement factor 5a receptor (C5aR). (elsevier.com)
  • The unregulated activity of the complement system decreases blood levels of another complement protein called C3, reducing the immune system's ability to fight infections. (medlineplus.gov)
  • We propose that unopposed complement activation is a risk factor for both immune and nonimmune forms of microvascular injuries in renal allografts. (nih.gov)
  • In this study we could show that the immune system and more specifically the complement system was dysregulated in a large fraction of patients. (diva-portal.org)
  • Development of neutralizing antibodies against therapeutic Factor VIII (FVIII) is the most serious complication of the treatment of hemophilia A. There is growing evidence to show the multifactorial origin of the anti-FVIII immune response, combining both genetic and environmental factors. (haematologica.org)
  • Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as in vivo complement depletion in FVIII-deficient mice, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII. (haematologica.org)
  • The purple sea urchin, Strongylocentrotus purpuratus, possesses a non-adaptive immune system including elements homologous to C3 and factor B (Bf) of the vertebrate complement system. (semanticscholar.org)
  • Our published work showed that fBR32Q influences C3 convertase formation, whereas fHV62I affects factor I cofactor activity. (csic.es)
  • The action of six different enzymes on the function and structure of Factor H was investigated by use of sodium dodecyl sulphate/polyacrylamide-gel electrophoresis, haemagglutination, two enzyme-linked immunosorbent assay systems and an assay for Factor I cofactor activity. (semanticscholar.org)
  • The 155-KDa glycoprotein complement factor H (FH) was first described in 1965 and soon emerged as a major soluble inhibitor of the complement system. (frontiersin.org)
  • These include interactions with the host complement system that may facilitate pathogen entry into cells and tissues, expression of molecules that defuse the effector complement components and complexes, and acquisition of host complement inhibitors to downregulate complement activity on the surface of the pathogen. (frontiersin.org)
  • 3 CRP elicits a multitude of effects on endothelial biology that favor a proatherosclerotic phenotype, such as decreasing NO release, 4 upregulating adhesion molecules, 5 stimulating vascular smooth muscle cell proliferation and migration, 6 and activating the complement system. (ahajournals.org)
  • The complement system has been shown to mediate renal ischemia-reperfusion (I/R) injury. (rug.nl)
  • Factor H is a major regulatory protein of the complement system. (acris-antibodies.com)
  • Interactions of the complement system with endotoxic lipopolysaccharide. (eurekamag.com)
  • Endotoxic lipopolysaccharide has recently been shown to fix large amounts of the complement components related to the biologic activities mediated by that system. (eurekamag.com)
  • Molecular sieve chromatography and sucrose density gradient ultracentrifugation demonstrated that the chemotactic factor was a relatively low molecular weight product (15,000-30,000) and as such different from previously scribed C' system-derived chemotactic factors. (eurekamag.com)
  • It plays a critical role in the homeostasis of the complement system in plasma and in the protection of bystander host cells and tissues from damage by complement activation. (ptglab.com)
  • demonstrated that M6 protein and other M serotypes bind factor H (fH), a regulatory protein of the complement system, resulting in reduced deposition of C3b on the streptococcal surface ( 15 ). (asm.org)
  • Coelomocytes express SpBf, a homologue of factor B, the second component in the sea urchin complement system. (semanticscholar.org)
  • Echinoderm immunity and the evolution of the complement system. (semanticscholar.org)
  • The ancestral complement system in sea urchins. (semanticscholar.org)
  • C3b and factor H: key components of the complement system. (mysciencework.com)
  • Human polyclonal and monoclonal IgG and IgM complement 3 nephritic factors: evidence for idiotypic commonality. (harvard.edu)
  • Structural and functional analysis of the complement component factor H with the use of different enzymes and monoclonal antibodies to factor H. (semanticscholar.org)
  • Atypical Hemolytic Uremic Syndrome Atypical hemolytic uremic syndrome is caused by complement overactivation. (wikipedia.org)
  • Defect in the factor H C terminus leads to a dramatically increased risk of atypical hemolytic uremic syndrome. (mysciencework.com)
  • Factor I is a glycoprotein heterodimer consisting of a disulfide linked heavy chain and light chain. (wikipedia.org)
  • Factor H is a glycoprotein consisting of a single polypeptide chain with a molecular mass of 150 kDa. (fishersci.com)
  • In this review we discuss current concepts on the physiological and pathophysiological roles of factor H in light of new data and recent developments in our understanding of the versatile roles of factor H as an inhibitor of complement activation and inflammation, as well as a mediator of cellular interactions. (mdpi.com)
  • To understand how miR-146a modulates inflammatory signaling in TLE, we investigated the role of interleukin-1β (IL-1β), miR-146a and human complement factor H (CFH) in the perpetuate inflammation in rat models of chronic TLE and U251 cells. (biologists.org)
  • In the present study, we examined the role of complement factor 3 (C3) in the development of PM-induced AHR and airway inflammation by comparing responses between C3-deficient (C3 -/- ) and wild-type mice. (elsevier.com)
  • a circulating inhibitor of complement activation) provides evidence that inflammation in general, and complement in particular, maybe causally involved in AMD. (ucl.ac.uk)
  • Since AMD and atherosclerosis share similar pathological features and risk factors, including a link with inflammation, an important question arises: is complement factor H (fH) a shared risk factor for both AMD and CHD? (ucl.ac.uk)
  • Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. (abcam.com)
  • The factor I light chain contains only the serine protease domain. (wikipedia.org)
  • It is an exceptionally specific protease and the only known protein substrate is factor B in complex with C3.3 Factor D protease activity is regulated by reversible conformational changes, which differs from the majority of serine proteases whose regulation involves either activation by processing of the zymogens or inactivation by binding of the inhibitors. (rndsystems.com)
  • Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb. (umassmed.edu)
  • Conventional protease inhibitors do not completely inactivate Factor I but they can do so if the enzyme is pre-incubated with its substrate: this supports the proposed rearrangement of the molecule upon binding to the substrate. (wikipedia.org)
  • 4. The activation of blood protease by streptococcal fibrinolysin is prevented by many reagents that remove or destroy midpiece of complement. (jimmunol.org)
  • We offer Complement Factor H Lysates for use in common research applications: Western Blot. (novusbio.com)
  • A mutation in complement factor B was associated with a case of C3 glomerulonephritis. (antikoerper-online.de)
  • Le Quintrec M, Lionet A, Kamar N, Karras A, Barbier S, Buchler M et al (2008) Complement mutation-associated de novo thrombotic microangiopathy following kidney transplantation. (springer.com)
  • Endotoxoids" deficient in complement-firing activity were also deficient in chemotactic factor generation. (eurekamag.com)
  • Chemotactic factor could not be generated by endotoxin in sera of mice congenitally deficient in the C'S component of complement, while chemotactic factor was generated by endotoxin in the sera of coisogenic mice with normal complement levels for that species. (eurekamag.com)
  • The chemotactic factor induced by endotoxin was heat stable and nondialyzable. (eurekamag.com)
  • In order to prevent damage to self cells and tissues and restrict overconsumption of the complement components, C3b molecules need to be controlled by factor H. Defect in C3 functions leads to compromised microbial defence and increased susceptibility to certain autoimmune diseases. (mysciencework.com)
  • Moreover, numerous pathogenic microbes and some tumor cells have developed the ability to exploit FH for complement evasion. (frontiersin.org)
  • 3. FH in complement evasion by microbes or tumor cells. (frontiersin.org)
  • Our findings imply that SdrE functions as a 'clamp' to capture CFH's C-terminal tail via a unique CDLL mechanism and sequesters CFH on the surface of S. aureus for complement evasion. (biochemj.org)
  • We therefore characterized the CFHR3 function and identified CFHR3 as a novel human complement regulator that inhibits C3 convertase activity. (uni-regensburg.de)
  • conducted a clinical trial of lampalizumab, an inhibitor of complement factor D, and noted a potential therapeutic effect on GA. (aao.org)
  • Factor H is an inhibitor of complement activation and promotes tumor growth in vivo ( 18 ). (aacrjournals.org)
  • COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis. (harvard.edu)
  • Staphylococcus epidermidis induces complement activation, tumor necrosis factor and interleukin-1, a shock-like state and tissue injury in rabbits without endotoxemia. (jci.org)
  • the activated complex of the fifth component of complement C5a induces chemotaxis in the case of polymorphonuclear leukocytes. (thefreedictionary.com)
  • The activated complex of the fifth, sixth, and seventh components of complement (C567), which induces chemotaxis of polymorphonuclear leukocytes. (thefreedictionary.com)
  • The 170 kDa protein was identified as the human complement protein Factor H. Human Factor H, isolated by a different method, was shown to bind specifically to L-selectin in the presence of CaCl 2 , and binding was inhibited by anti-L-selectin antibodies, fucoidan and lipopolysaccharide. (biochemj.org)
  • Streptococcus pyogenes evades complement by binding the complement-regulatory protein factor H (fH) via the central conserved C-repeat region of M protein. (asm.org)
  • 11 ) also confirmed that the acquisition of complement regulatory protein factor H (FH) by GAS contributes to the bacterium's capacity to evade phagocytosis by polymorphonuclear leukocytes (PMNs). (asm.org)
  • Immunostaining for membrane attack complex of complement is related to cell necrosis in fulminant and acute hepatitis," Gastroenterology , vol. 108, no. 2, pp. 495-504, 1995. (hindawi.com)
  • Furthermore, a study, using well-characterized human donor eyes, showed that AMD disease severity and complement genotypes are associated with complement activation in the eye. (molvis.org)
  • The molecular genetics and polymorphism of C2 and factor B". British Medical Bulletin. (wikipedia.org)
  • These results suggest that exposure to PM may induce AHR through activation of complement factor 3 in the airways. (elsevier.com)
  • In addition to its canonical role in complement regulation, several other functions of FH have been discovered and a large number of binding partners have been identified. (frontiersin.org)
  • The regulatory role of Factor H is essential because C3bBb is not only a C5 convertase but a C3 convertaseand so has a positive feedback effect, potentially consuming the entire C3 pool if unregulated. (acris-antibodies.com)
  • The species-specific pattern of viability and/or lysis is highly consistent with the pattern of reservoir competence of hosts for B. burgdorferi sensu lato, suggesting a key role of complement in the global ecology of Lyme borreliosis. (asm.org)
  • 9 This is supported by the presence of activated complement components in atherosclerotic plaques, such as the membrane attack complex (MAC, C5b-9), which promotes cellular activation, upregulates adhesion molecules, stimulates chemokine secretion, and can cause cell lysis. (ahajournals.org)
  • When incorporated into the membrane of rabbit erythrocytes, human DAF inhibited their lysis by human complement. (rupress.org)
  • In addition, CFHR3 and CFHR1 compete with factor H for binding to the central complement component C3. (uni-regensburg.de)