A specific mannose-binding member of the collectin family of lectins. It binds to carbohydrate groups on invading pathogens and plays a key role in the MANNOSE-BINDING LECTIN COMPLEMENT PATHWAY.
A class of C-type lectins that target the carbohydrate structures found on invading pathogens. Binding of collectins to microorganisms results in their agglutination and enhanced clearance. Collectins form trimers that may assemble into larger oligomers. Each collectin polypeptide chain consists of four regions: a relatively short N-terminal region, a collagen-like region, an alpha-helical coiled-coil region, and carbohydrate-binding region.
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.
A hexose or fermentable monosaccharide and isomer of glucose from manna, the ash Fraxinus ornus and related plants. (From Grant & Hackh's Chemical Dictionary, 5th ed & Random House Unabridged Dictionary, 2d ed)
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.
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.
A subclass of lectins that are specific for CARBOHYDRATES that contain MANNOSE.
Proteins that share the common characteristic of binding to carbohydrates. Some ANTIBODIES and carbohydrate-metabolizing proteins (ENZYMES) also bind to carbohydrates, however they are not considered lectins. PLANT LECTINS are carbohydrate-binding proteins that have been primarily identified by their hemagglutinating activity (HEMAGGLUTININS). However, a variety of lectins occur in animal species where they serve diverse array of functions through specific carbohydrate recognition.
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.
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
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.
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.
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 class of animal lectins that bind specifically to beta-galactoside in a calcium-independent manner. Members of this class are distiguished from other lectins by the presence of a conserved carbohydrate recognition domain. The majority of proteins in this class bind to sugar molecules in a sulfhydryl-dependent manner and are often referred to as S-type lectins, however this property is not required for membership in this class.
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 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.
A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.
Serum serine proteases which participate in COMPLEMENT ACTIVATION. They are activated when complexed with the MANNOSE-BINDING LECTIN, therefore also known as Mannose-binding protein-Associated Serine Proteases (MASPs). They cleave COMPLEMENT C4 and COMPLEMENT C2 to form C4b2a, the CLASSICAL PATHWAY C3 CONVERTASE.
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.
Protein or glycoprotein substances of plant origin that bind to sugar moieties in cell walls or membranes. Some carbohydrate-metabolizing proteins (ENZYMES) from PLANTS also bind to carbohydrates, however they are not considered lectins. Many plant lectins change the physiology of the membrane of BLOOD CELLS to cause agglutination, mitosis, or other biochemical changes. They may play a role in plant defense mechanisms.
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).
Transport proteins that carry specific substances in the blood or across cell membranes.
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.
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 multifunctional galactin initially discovered as a macrophage antigen that binds to IMMUNOGLOBULIN E, and as 29-35-kDa lectin that binds LAMININ. It is involved in a variety of biological events including interactions with galactose-containing glycoconjugates, cell proliferation, CELL DIFFERENTIATION, and APOPTOSIS.
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.
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 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.
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.
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.
Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.
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 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.
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).
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.
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.
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.
Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.
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 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.
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.
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.
Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.
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.
Proteins that bind to particles and cells to increase susceptibility to PHAGOCYTOSIS, especially ANTIBODIES bound to EPITOPES that attach to FC RECEPTORS. COMPLEMENT C3B may also participate.
The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.
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).
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.
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.
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.
A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).
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.
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).
Phosphoric acid esters of mannose.
A receptor that is specific for IGF-II and mannose-6-phosphate. The receptor is a 250-kDa single chain polypeptide which is unrelated in structure to the type 1 IGF receptor (RECEPTOR, IGF TYPE 1) and does not have a tyrosine kinase domain.
The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).
The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.
The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.
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.

Mannan binding lectin in febrile adults: no correlation with microbial infection and complement activation. (1/88)

AIMS: To study the role of the mannan binding lectin (MBL) pathway of complement activation in the host defence to microbial infection in vivo, and the role of MBL in infectious mortality in non-selected patients. METHODS: A prospective observational study on 177 hospitalised medical patients with new onset fever. The presence, origin, and microbial cause of infection, the circulating MBL and complement activation product 3a (C3a), and the 28 day hospital course were determined. RESULTS: The patients had median MBL values similar to healthy blood donors: 18% of the patients and 14% of the blood donors had MBL deficiency, with values below 0.1 microg/ml. Median C3a was higher in patients with microbiologically confirmed infection than in those without, whereas there was no difference in MBL values or frequency of deficiency among patient groups with or without positive local cultures or bacteraemia. The mortality rate was 8% and the outcome groups did not differ in MBL. In febrile adults hospitalised in internal medicine wards, microbial infection induces complement activation, independently of MBL. CONCLUSIONS: The results argue against a predominant role for the MBL pathway of complement activation and a deficiency of MBL predisposing to serious and invasive microbial infection in non-selected adults.  (+info)

Human mannose-binding lectin and L-ficolin function as specific pattern recognition proteins in the lectin activation pathway of complement. (2/88)

The innate immune response in vertebrates and invertebrates requires the presence of pattern recognition receptors or proteins that recognize microbial cell components including lipopolysaccharide, bacterial peptidoglycan (PGN), and fungal 1,3-beta-D-glucan. We reported previously that PGN and 1,3-beta-D-glucan recognition proteins from insect hemolymph were able to induce the activation of the prophenoloxidase-activating system, one of the major invertebrate innate immune reactions. The goal of this study was to characterize the biochemical properties and effects of the human counterparts of these molecules. Soluble pattern recognition proteins were purified from human serum and identified as human mannose-binding lectin (MBL) and L-ficolin. The use of specific microbial cell component-coupled columns demonstrated that MBL and L-ficolin bind to PGN and 1,3-beta-D-glucan, respectively. Purified MBL and L-ficolin were associated with MBL-associated serine proteases-1 and -2 (MASPs) and small MBL-associated protein as determined by Western blot analysis. Finally, the binding of purified MBL/MASP and L-ficolin/MASP complexes to PGN and 1,3-beta-D-glucan, respectively, resulted in the activation of the lectin-complement pathway. These results indicate that human PGN and 1,3-beta-D-glucan recognition proteins function as complement-activating lectins.  (+info)

Role of mannose-binding lectin in the innate defense against Candida albicans: enhancement of complement activation, but lack of opsonic function, in phagocytosis by human dendritic cells. (3/88)

Mannose-binding lectin (MBL) is a serum collectin believed to be of importance in innate immunity. We have investigated the role of MBL in the first-line defense against Candida albicans, an opportunistic fungal pathogen. MBL bound C. albicans via its lectin domain, resulting in agglutination of the organisms upon their outgrowth of hyphae. In a human in vitro MBL system, deposition of C4 fragments on C. albicans was increased when exogenous MBL was added to serum samples from MBL-deficient individuals. Similar enhancement of deposition of iC3b also was observed. MBL and enhanced opsonic C3 fragments mediated by MBL did not facilitate opsonophagocytosis of the organisms by monocyte-derived dendritic cells (DCs). However, MBL was found to inhibit the growth of C. albicans independently of complement activation, although, with complement activation, further inhibition was observed. We concluded that MBL plays an important role in the first-line defense against C. albicans without the need for opsonophagocytosis by DCs, in which a direct interaction of MBL with C. albicans results in agglutination and accelerated complement activation via the lectin pathway, leading to inhibition of growth.  (+info)

Role of L-ficolin/mannose-binding lectin-associated serine protease complexes in the opsonophagocytosis of type III group B streptococci. (4/88)

Serotype III group B streptococci (GBS) are a common cause of neonatal sepsis and meningitis. Although deficiency in maternal capsular polysaccharide (CPS)-specific IgG correlates with susceptibility of neonates to the GBS infection, serum deficient in CPS-specific IgG mediates significant opsonophagocytosis. This IgG-independent opsonophagocytosis requires activation of the complement pathway, a process requiring the presence of both Ca(2+) and Mg(2+), and is significantly reduced by chelating Ca(2+) with EGTA. In these studies, we defined a role of L-ficolin/mannose-binding lectin-associated serine protease (MASP) complexes in Ca(2+)-dependent, Ab-independent opsonophagocytosis of serotype III GBS. Incubation of GBS with affinity-purified L-ficolin/MASP complexes and C1q-depleted serum deficient in CPS-specific Ab supported opsonophagocytic killing, and this killing was inhibited by fluid-phase N-acetylglucosamine, the ligand for L-ficolin. Binding of L-ficolin was proportional to the CPS content of individual strains, and opsonophagocytic killing and C4 activation were inhibited by fluid-phase CPS, suggesting that L-ficolin binds to CPS. Sialic acid is known to inhibit alternative complement pathway activation, and, as expected, the bactericidal index (percentage of bacteria killed) for individual strains was inversely proportional to the sialic acid content of the CPS, and L-ficolin-initiated opsonophagocytic killing was significantly increased by addition of CPS-specific IgG2, which increased activation of the alternative pathway. We conclude that binding of L-ficolin/MASP complexes to the CPS generates C3 convertase C4b2a, which deposits C3b on GBS. C3b deposited by this lectin pathway forms alternative pathway C3 convertase C3bBb whose activity is enhanced by CPS-specific IgG2, leading to increased opsonophagocytic killing by further deposition of C3b on the GBS.  (+info)

Deficiency of the mannan-binding lectin pathway of complement and poor outcome in cystic fibrosis: bacterial colonization may be decisive for a relationship. (5/88)

In cystic fibrosis (CF) prognosis concerning lung damage development is highly variable and difficult to predict. Mannan-binding lectin (MBL) deficiency has been reported to be associated with poor outcome in CF lung disease. MBL is a recognition molecule of the MBL pathway of the complement system and is encoded by a gene characterized by a high degree of polymorphism. Some genotypes result in low serum concentrations of MBL. MBL-associated serine protease 2 (MASP-2) is another protein belonging to the MBL pathway. A mutation resulting in low levels of MASP-2 in serum has been described recently. In the present study, 112 CF patients aged 4-54 years were investigated for MBL and MASP-2 genotypes, serum levels of MBL and MASP-2 and the MBL pathway function in serum. No correlation to reduced lung function or need for lung transplantation was seen, either for MBL deficiency, MASP-2 gene mutation or reduced MBL pathway function. However, in the 27 patients colonized with Staphylococcus aureus, MBL-deficient genotypes were associated with decreased lung function. As expected, MBL pathway function in serum was reduced both in MBL-deficient patients and in patients carrying a mutant MASP-2 allele. An unexpected finding was that CF patients had higher serum levels of MBL than healthy controls when corrected for MBL genotype. In conclusion, MBL pathway function was affected both by MBL and by MASP-2 genotypes. However, MBL or MASP-2 levels in serum did not affect the clinical outcome in the cohort of CF patients studied.  (+info)

Composition of the lectin pathway of complement in Gallus gallus: absence of mannan-binding lectin-associated serine protease-1 in birds. (6/88)

The lectin pathway of complement is activated by multimolecular complexes that recognize and bind to microbial polysaccharides. These complexes comprise a multimeric carbohydrate recognition subunit (either mannan-binding lectin (MBL) or a ficolin), three MBL-associated serine proteases (MASP-1, -2, and -3), and MAp19 (a truncated product of the MASP-2 gene). In this study we report the cloning of chicken MASP-2, MASP-3, and MAp19 and the organization of their genes and those for chicken MBL and a novel ficolin. Mammals usually possess two MBL genes and two or three ficolin genes, but chickens have only one of each, both of which represent the undiversified ancestors of the mammalian genes. The primary structure of chicken MASP-2 is 54% identical with those of the human and mouse MASP-2, and the organization of its gene is the same as in mammals. MASP-3 is even more conserved; chicken MASP-3 shares approximately 75% of its residues with human and Xenopus MASP-3. It is more widely expressed than other lectin pathway components, suggesting a possible function of MASP-3 different from those of the other components. In mammals, MASP-1 and MASP-3 are alternatively spliced products of a single structural gene. We demonstrate the absence of MASP-1 in birds, possibly caused by the loss of MASP-1-specific exons during phylogeny. Despite the lack of MASP-1-like enzymatic activity in sera of chicken and other birds, avian lectin pathway complexes efficiently activate C4.  (+info)

Gastrointestinal ischemia-reperfusion injury is lectin complement pathway dependent without involving C1q. (7/88)

Complement activation plays an important role in local and remote tissue injury associated with gastrointestinal ischemia-reperfusion (GI/R). The role of the classical and lectin complement pathways in GI/R injury was evaluated using C1q-deficient (C1q KO), MBL-A/C-deficient (MBL-null), complement factor 2- and factor B-deficient (C2/fB KO), and wild-type (WT) mice. Gastrointestinal ischemia (20 min), followed by 3-h reperfusion, induced intestinal and lung injury in C1q KO and WT mice, but not in C2/fB KO mice. Addition of human C2 to C2/fB KO mice significantly restored GI/R injury, demonstrating that GI/R injury is mediated via the lectin and/or classical pathway. Tissue C3 deposition in C1q KO and WT, but not C2/fB KO, mice after GI/R demonstrated that complement was activated in C1q KO mice. GI/R significantly increased serum alanine aminotransferase, gastrointestinal barrier dysfunction, and neutrophil infiltration into the lung and gut in C1q KO and WT, but not C2/fB KO, mice. MBL-null mice displayed little gut injury after GI/R, but lung injury was present. Addition of recombinant human MBL (rhuMBL) to MBL-null mice significantly increased injury compared with MBL-null mice after GI/R and was reversed by anti-MBL mAb treatment. However, MBL-null mice were not protected from secondary lung injury after GI/R. These data demonstrate that C2 and MBL, but not C1q, are necessary for gut injury after GI/R. Lung injury in mice after GI/R is MBL and C1q independent, but C2 dependent, suggesting a potential role for ficolins in this model.  (+info)

Mannose-binding lectin is a regulator of inflammation that accompanies myocardial ischemia and reperfusion injury. (8/88)

The mannose-binding lectin (MBL), a circulating pattern recognition molecule, recognizes a wide range of infectious agents with resultant initiation of the complement cascade in an Ab-independent manner. MBL recognizes infectious non-self and altered self in the guise of apoptotic and necrotic cells. In this study, we demonstrate that mice lacking MBL, and hence are devoid of MBL-dependent lectin pathway activation but have fully active alternative and classical complement pathways, are protected from cardiac reperfusion injury with resultant preservation of cardiac function. Significantly, mice that lack a major component of the classical complement pathway initiation complex (C1q) but have an intact MBL complement pathway, are not protected from injury. These results suggest that the MBL-dependent pathway of complement activation is a key regulator of myocardial reperfusion ischemic injury. MBL is an example of a pattern recognition molecule that plays a dual role in modifying inflammatory responses to sterile and infectious injury.  (+info)

Explanation: Genetic predisposition to disease is influenced by multiple factors, including the presence of inherited genetic mutations or variations, environmental factors, and lifestyle choices. The likelihood of developing a particular disease can be increased by inherited genetic mutations that affect the functioning of specific genes or biological pathways. For example, inherited mutations in the BRCA1 and BRCA2 genes increase the risk of developing breast and ovarian cancer.

The expression of genetic predisposition to disease can vary widely, and not all individuals with a genetic predisposition will develop the disease. Additionally, many factors can influence the likelihood of developing a particular disease, such as environmental exposures, lifestyle choices, and other health conditions.

Inheritance patterns: Genetic predisposition to disease can be inherited in an autosomal dominant, autosomal recessive, or multifactorial pattern, depending on the specific disease and the genetic mutations involved. Autosomal dominant inheritance means that a single copy of the mutated gene is enough to cause the disease, while autosomal recessive inheritance requires two copies of the mutated gene. Multifactorial inheritance involves multiple genes and environmental factors contributing to the development of the disease.

Examples of diseases with a known genetic predisposition:

1. Huntington's disease: An autosomal dominant disorder caused by an expansion of a CAG repeat in the Huntingtin gene, leading to progressive neurodegeneration and cognitive decline.
2. Cystic fibrosis: An autosomal recessive disorder caused by mutations in the CFTR gene, leading to respiratory and digestive problems.
3. BRCA1/2-related breast and ovarian cancer: An inherited increased risk of developing breast and ovarian cancer due to mutations in the BRCA1 or BRCA2 genes.
4. Sickle cell anemia: An autosomal recessive disorder caused by a point mutation in the HBB gene, leading to defective hemoglobin production and red blood cell sickling.
5. Type 1 diabetes: An autoimmune disease caused by a combination of genetic and environmental factors, including multiple genes in the HLA complex.

Understanding the genetic basis of disease can help with early detection, prevention, and treatment. For example, genetic testing can identify individuals who are at risk for certain diseases, allowing for earlier intervention and preventive measures. Additionally, understanding the genetic basis of a disease can inform the development of targeted therapies and personalized medicine."


There are two main types of hemolysis:

1. Intravascular hemolysis: This type occurs within the blood vessels and is caused by factors such as mechanical injury, oxidative stress, and certain infections.
2. Extravascular hemolysis: This type occurs outside the blood vessels and is caused by factors such as bone marrow disorders, splenic rupture, and certain medications.

Hemolytic anemia is a condition that occurs when there is excessive hemolysis of RBCs, leading to a decrease in the number of healthy red blood cells in the body. This can cause symptoms such as fatigue, weakness, pale skin, and shortness of breath.

Some common causes of hemolysis include:

1. Genetic disorders such as sickle cell anemia and thalassemia.
2. Autoimmune disorders such as autoimmune hemolytic anemia (AIHA).
3. Infections such as malaria, babesiosis, and toxoplasmosis.
4. Medications such as antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), and blood thinners.
5. Bone marrow disorders such as aplastic anemia and myelofibrosis.
6. Splenic rupture or surgical removal of the spleen.
7. Mechanical injury to the blood vessels.

Diagnosis of hemolysis is based on a combination of physical examination, medical history, and laboratory tests such as complete blood count (CBC), blood smear examination, and direct Coombs test. Treatment depends on the underlying cause and may include supportive care, blood transfusions, and medications to suppress the immune system or prevent infection.

The lectin pathway of complement is activated by mannose-binding lectin (MBL) which binds to N-acetylglucosamine. N- ... "Involvement of lectin pathway activation in the complement killing of Giardia intestinalis". Biochemical and Biophysical ... The classical pathway of complement is activated by antibodies specific against Giardia. Antibodies inhibit parasite ... replication and also induce parasite death via the classical pathway of complement.[citation needed] Infection with Giardia ...
"The emerging roles of mannose-binding lectin-associated serine proteases (MASPs) in the lectin pathway of complement and beyond ... Mannose-binding protein-associated serine protease are serine proteases involved in the complement system. Types include: MASP1 ... "Molecular characterization of a novel serine protease involved in activation of the complement system by mannose-binding ... MASP2 mannan-binding lectin Dobó, József; Pál, Gábor; Cervenak, László; Gál, Péter (November 2016). " ...
The lectin pathway starts with mannose-binding lectin (MBL) or ficolin binding to certain sugars. In this pathway, mannose- ... Classical complement pathway Alternative complement pathway Mannan-binding lectin Wallis R, Mitchell DA, Schmid R, Schwaeble WJ ... The lectin pathway or lectin complement pathway is a type of cascade reaction in the complement system, similar in structure to ... In contrast to the classical complement pathway, the lectin pathway does not recognize an antibody bound to its target. ...
The lectin pathway is homologous to the classical pathway, but with the opsonin, mannose-binding lectin (MBL), and ficolins, ... the classical complement pathway, the alternative complement pathway, and the lectin pathway. The alternative pathway accounts ... The mannose-binding lectin pathway can be activated by C3 hydrolysis or antigens without the presence of antibodies (non- ... Alternative Complement Pathway) Inflammation - by attracting macrophages and neutrophils. (Lectin pathway) Most of the proteins ...
Collectins (e.g. mannose-binding lectin and surfactant protein A) bind the altered surface sugars on apoptotic cell and enable ... components of complement pathways (e.g. C1q, C3b) and other molecules found in extracellular space. ... lectins (binding the altered sugars), the receptor tyrosine kinase MER (recognizing GAS-6), LRP1 (interacts with calreticulin ... Besides complement particles C1q and C3b which help to opsonize the apoptotic cells, also thrombospondin, pentraxins (C- ...
... complement pathway, classical MeSH G04.610.255.849 - complement pathway, mannose-binding lectin MeSH G04.610.270.070 - antibody ... binding sites, antibody MeSH G04.610.143.281 - cross reactions MeSH G04.610.143.545 - hemolysis MeSH G04.610.143.612 - ...
The lectin pathway is activated when pattern-recognition receptors, like mannan-binding lectin or ficolins, recognize and bind ... These bound receptors then complex with Mannose-Binding Lectin-Associated Serine Proteases (MASPs), which have proteolytic ... Degn, Søren E.; Thiel, Steffen; Jensenius, Jens C. (2007-01-01). "New perspectives on mannan-binding lectin-mediated complement ... The classical pathway of complement activation is initiated when the C1 complex, made up of C1r and C1s serine proteases, ...
"Selective inhibition of the lectin pathway of complement with phage display selected peptides against mannose-binding lectin- ... MASP1 (protein) Mannan-binding lectin Mannan-binding lectin pathway (lectin pathway) GRCh38: Ensembl release 89: ... Petersen SV, Thiel S, Jensenius JC (2001). "The mannan-binding lectin pathway of complement activation: biology and disease ... Mannan-binding lectin serine protease 2 also known as mannose-binding protein-associated serine protease 2 (MASP-2) is an ...
"Selective inhibition of the lectin pathway of complement with phage display selected peptides against mannose-binding lectin- ... Petersen SV, Thiel S, Jensenius JC (August 2001). "The mannan-binding lectin pathway of complement activation: biology and ... MASP-1 is a serine protease that functions as a component of the lectin pathway of complement activation. The complement ... the mannose-binding lectin and the ficolins. This protein is directly involved in complement activation because MASP-1 ...
The lectin pathway is triggered when mannose-binding lectin (MBL) or ficolin aka specific pattern recognition receptors bind to ... Each of the three pathways ensures that complement will still be functional if one pathway ceases to work or a foreign invader ... consists of three pathways that are activated in distinct ways. The classical pathway is triggered when IgG or IgM is bound to ... This binding results in the activation of a signaling pathway which allows for the transcription factor NF-κB to enter the ...
... and the lectin pathway. One way the most-recently discovered lectin pathway is activated is through mannose-binding lectin ... Binding of MBL to a micro-organism results in activation of the lectin pathway of the complement system. Another important ... Mannose-binding lectin (MBL), also called mannan-binding lectin or mannan-binding protein (MBP), is a lectin that is ... The complement system can be activated through three pathways: the classical pathway, the alternative pathway, ...
... the classical complement pathway, the alternate complement pathway, and the mannose-binding lectin pathway. The classical ... 12 of which are directly involved in the complement pathways. The complement system is involved in the activities of both ... The complement system consists of more than 35 soluble and cell-bound proteins, ... The B cell waits for a helper T cell (TH) to bind to the complex. This binding will activate the TH cell, which then releases ...
... hence preventing cleavage of C4 and C2 mannan-binding lectin lectin pathway Degn SE, Hansen AG, Steffensen R, Jacobsen C, ... Mannose-binding lectin-associated protein of 44 kDa (MAp44) is a protein arising from the human MASP1 gene. MASP-1, MASP-3 and ... protein associated with pattern recognition molecules of the complement system and regulating the lectin pathway of complement ... MAp44 has been suggested to act as a competitive inhibitor of lectin pathway activation, by displacing MASP-2 from MBL, ...
IgM antibodies also bind to PC. Collectin molecules such as mannose-binding lectin (MBL), surfactant protein A (SP-A), and SP-D ... C3b can spontaneously bind to pathogen surfaces through the alternative complement pathway. Furthermore, pentraxins can ... Mannose-binding lectins, or ficolins, along with pentraxins and collectins are able to recognize certain types of carbohydrates ... Complement proteins involved in innate opsonization include C4b, C3b and iC3b. In the alternative pathway of complement ...
"Mannose-binding lectin deficiency". Genetics Home Reference. U.S. National Library of Medicine. v t e (Articles with short ... MBL deficiency is a pathology of the innate immune system involving Mannan-binding lectin pathway components such as MBL2. It ... description, Short description matches Wikidata, Short description is different from Wikidata, Complement deficiency, All stub ...
... in response to binding to CRP or immunoglobulin, and in the lectin pathway it is driven by mannose binding lectin and its ... lectin, and alternative pathways. Cleavage of complement C3 by a free floating convertase, thrombin, plasmin or even a ... Binding β1H to C3b increases C3bINA binding, while factor B binding prevents C3bINA binding and is competitive with β1H binding ... C4 binding protein (C4BP) interferes with the assembly of the membrane-bound C3 convertase of the classical pathway. C4BP is a ...
Both genes encode proteins of the lectin complement pathway, which plays a role in the complement system of innate, or non- ... "Mannose binding lectin (MBL) and HIV". Molecular Immunology. 42 (2): 145-52. doi:10.1016/j.molimm.2004.06.015. PMID 15488604. ... The protein is a type of connectin called a mannan-binding lectin, which plays a role in innate immunity by binding to ... "Mutations in lectin complement pathway genes COLEC11 and MASP1 cause 3MC syndrome". Nature Genetics. 43 (3): 197-203. doi: ...
"Entrez Gene: LMAN1 lectin, mannose-binding, 1". Khoriaty R, Vasievich MP, Ginsburg D (July 2012). "The COPII pathway and ... 1994). "Complement activation upon binding of mannan-binding protein to HIV envelope glycoproteins". AIDS. 7 (10): 1307-13. doi ... 2003). "High mannose glycans and sialic acid on gp120 regulate binding of mannose-binding lectin (MBL) to HIV type 1". AIDS Res ... The protein is a mannose-specific lectin and is a member of a novel family of plant lectin homologs in the secretory pathway of ...
The extracellular domain of the receptors contains a lectin-like complement-binding domain. Recognition by complement receptors ... Mannose and other pathogen-associated sugars, such as fucose, are recognised by the mannose receptor. Eight lectin-like domains ... Non-opsonic receptors include lectin-type receptors, Dectin receptor, or scavenger receptors. Some phagocytic pathways require ... Among these are receptors that recognise the Fc part of bound IgG antibodies, deposited complement or receptors, that recognise ...
... s are linked with activation of lectin pathway of complement activation. At the beginning, there is a binding of ... Nine types of collectins have been defined: MBL = mannan-binding lectin (mannose-binding lectin) SP-A = surfactant protein A SP ... "The mannan-binding lectin-associated serine proteases (MASPs) and MAp19: four components of the lectin pathway activation ... "The mannan-binding lectin-associated serine proteases (MASPs) and MAp19: four components of the lectin pathway activation ...
Lectin: starts when lectins bind to mannose on bacteria Elements of the complement cascade can be found in many non-mammalian ... "Activation of the human complement alternative pathway by Listeria monocytogenes: evidence for direct binding and proteolysis ... The three different complement systems are classical, alternative and lectin. Classical: starts when antibody binds to bacteria ... The complement system is a biochemical cascade of the immune system that helps, or "complements", the ability of antibodies to ...
Matsushita M, Fujita T (December 1992). "Activation of the classical complement pathway by mannose-binding protein in ... mannose-binding lectin-associated serine protease-2, p100, mannan-binding lectin-associated serine peptidase 2) is an enzyme. ... and after Arg76 in complement component C4 (-Gly-Leu-Gln-Arg-Ala-Leu-Glu-Ile) This mannan-binding lectin (MBL) recognizes ... "A second serine protease associated with mannan-binding lectin that activates complement". Nature. 386 (6624): 506-10. doi: ...
... and activate the lectin pathway of the complement cascade. Specifically, ficolins bind to acetyl groups present in certain ... they initiate the proteolytic complement cascade, facilitated by the mannose-binding protein-associated serine proteases (MASPs ... "Lectin-complement pathway molecules are decreased in patients with cirrhosis and constitute the risk of bacterial infections". ... that bind to acetyl groups present in the carbohydrates of bacterial surfaces and mediate activation of the lectin pathway of ...
... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... TLR1 TLR2 TLR3 TLR4 TLR5 TLR6 TLR7 TLR8 TLR9 TLR10 TLR11 TLR12 TLR13 C-type lectin receptors (CLRs) Group 1 CLRs - Mannose ... C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding lectin Alternative complement pathway Factor B Factor D ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ...
Examples include the genes coding for the proteins TNFα, mannan-binding lectin, CTLA4, TGFβ, DC-SIGN, PLCE1, and particular ... the C-type lectins called DC-SIGN, mannose receptor and CLEC5A. DC-SIGN, a non-specific receptor for foreign material on ... October 2019). "Carica papaya extract in dengue: a systematic review and meta-analysis". BMC Complement Altern Med (Review). 19 ... through the innate immune system by augmenting the production of a large group of proteins mediated by the JAK-STAT pathway. ...
Lectin mannose binding 2 LNCR3 encoding protein Lung cancer susceptibility 3 LPCAT1: Lysophosphatidylcholine acyltransferase 1 ... Complement C1q tumor necrosis factor-related protein 3 C5orf45: Chromosome 5 open reading frame 45 CPLANE1: Ciliogenesis And ... encodes G protein-coupled receptor protein signaling pathway YIPF5: Yip1 domain family member 5 YTHDC2: encoding protein YTH ... Purine-rich element-binding protein A PWWP2A: encoding protein PWWP domain containing 2A RANBP3L: encoding protein RAN binding ...
Once bound to the ligands MBL and Ficolin oligomers recruit MASP1 and MASP2 and initiate the lectin pathway of complement ... Dommett RM, Klein N, Turner MW (September 2006). "Mannose-binding lectin in innate immunity: past, present and future". Tissue ... One very important collectin is mannan-binding lectin (MBL), a major PRR of the innate immune system that binds to a wide range ... It recognizes and binds to repeated mannose units on the surfaces of infectious agents and its activation triggers endocytosis ...
IgM can bind complement component C1 and activate the classical pathway, leading to opsonization of antigens and cytolysis. IgM ... lectin binding, various chromatographic systems and enzymatic sensitivity (reviewed in). The structure of the oligosaccharides ... at each site varies in detail, and the predominant oligosaccharides - biantennary, triantennary, high mannose-differ among the ... This binding depends on J chain. Two other Fc receptors that bind IgM-Fcα/µ-R and Fcµ-R -- have been detected. Fcα/µ-R, like ...
... can "hide" mannose antigens on the surface of host cells or bacteria from mannose-binding lectin.[citation needed] ... A recent genome level study examined a large set of sequenced microbial genomes, which indicated that biosynthetic pathways to ... This prevents activation of complement. Sialic acid in the form of polysialic acid is an unusual posttranslational modification ... Mandal, C. (1990). "Sialic acid binding lectins". Experientia. 46 (5): 433-441. doi:10.1007/BF01954221. PMID 2189746. S2CID ...
"Association of Mannose-Binding Lectin Gene Polymorphism but Not of Mannose-Binding Serine Protease 2 with Chronic Severe Aortic ... it triggers the activation of multiple pathways that stimulate further pro-inflammatory cytokine secretion. Mannose-binding ... Kaplan, MH; Bolande, R; Rakita, L; Blair, J (1964). "Presence of Bound Immunoglobulins and Complement in the Myocardium in ... This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and ...
... an EGF-like domain and a complement-binding protein-like domains (same as complement regulatory proteins: CRP) having short ... The extracellular region of P-selectin is composed of three different domains like other selectin types; a C-type lectin-like ... Among them, the inhibitory activity of semisynthetic sulfated tri mannose C-C-linked dimers (STMCs) to P-selectin was shown by ... contains the signal for sorting into the regulated secretory pathway". Mol. Biol. Cell. 3 (3): 309-21. doi:10.1091/mbc.3.3.309 ...
"Two lineages of mannose-binding lectin-associated serine protease (MASP) in vertebrates". Journal of Immunology. 161 (9): 4924- ... part of the lectin pathway GRCh38: Ensembl release 89: ENSG00000182326 - Ensembl, May 2017 GRCm38: Ensembl release 89: ... Complement component 1s (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... Busby TF, Ingham KC (May 1990). "NH2-terminal calcium-binding domain of human complement C1s- mediates the interaction of C1r- ...
... via sugar-binding proteins called lectins, which recognize specific carbohydrate moieties. Glycosylation is an important ... A mannose sugar is added to the first tryptophan residue in the sequence W-X-X-W (W indicates tryptophan; X is any amino acid ... disorders of lipid glycosylation and disorders of other glycosylation pathways and of multiple glycosylation pathways. No ... the first crystal structure of a protein containing this type of glycosylation was determined-that of human complement ...
Lectins, a group of proteins which bind different glycans, are often used to detect these lipophosphoglycan variants. For ... Hybrid progeny are formed that have full genomic complements from both parents. Mating only occurs in the sand fly vector, and ... 2016). "Leishmania uses Mincle to target an inhibitory ITAM signaling pathway in dendritic cells that dampens adaptive immunity ... and a phosphorylated galactose-mannose, with a termination in a neutral cap. Not only do these parasites develop ...
The complement pathway is composed of several subset pathways: the lectin/mannose pathway, alternative pathway and the ... macrophages which are unable to bind the Fc portion of the C1q antibody), leading to further complications. This seems to be ... classical complement pathway). In brief, the crucial role of C1q in the pathway is its importance as the first protein to start ... in the complement pathway) named C1-inhibitor. The inhibition of C1-inhibitor leads to over-activation of the complement ...
... can infect dendritic cells (DCs) by this CD4-CCR5 route, but another route using mannose-specific C-type lectin receptors ... HIV-1 will bind to any appropriate RNA. HIV-2 will preferentially bind to the mRNA that was used to create the Gag protein ... Together, the cDNA and its complement form a double-stranded viral DNA that is then transported into the cell nucleus. The ... Hiscott J, Kwon H, Génin P (2001). "Hostile takeovers: viral appropriation of the NF-kB pathway". Journal of Clinical ...
... classical-complement-pathway C3/C5 convertase EC 3.4.21.45: complement factor I EC 3.4.21.46: complement factor D EC 3.4.21.47 ... mannan-binding lectin-associated serine protease-2 EC 3.4.21.105: rhomboid protease EC 3.4.21.106: hepsin EC 3.4.21.107: ... dolichylphosphate-mannose phosphodiesterase EC 3.1.4.50: glycosylphosphatidylinositol phospholipase D EC 3.1.4.51: glucose-1- ... classical-complement-pathway C3/C5 convertase EC 3.4.21.45: complement factor I EC 3.4.21.46: complement factor D EC 3.4.21.47 ...
These mechanisms include the lectin complement pathway and coagulation enzyme-like activities on both pathogens. Fur-thermore, ... a mammalian lectin, is a pattern recognition molecule of the innate immune system and recognizes carbo-hydrates that are ... Enhancement of complement activation and opsonophagocytosis by complexes of mannose-binding lectin with mannose-binding lectin- ... Binding of mannose-binding lectin to fructosamines: a potential link be-tween hyperglycaemia and complement activation in ...
C1-INH also blocks activation of the lectin pathway by binding to mannose-binding lectin-associated serine proteases (MASPs). ... Within the complement system, C1-INH blocks the activation of C1 and the rest of the classic complement pathway by binding to ... Deficiencies in C1-INH allow unchecked activation of the classic complement pathway and other biochemical systems including the ... Other biochemical pathways in which C1-INH is active, such as those for fibrinolysis and clotting, also function relatively ...
Factor H (FH), a major soluble complement inhibitor, binds to dead cells and inhibits excessive complement activation on their ... of the FHRs with pentraxins resulted in enhanced activation of both the classical and the alternative complement pathways on ... Factor H (FH), a major soluble complement inhibitor, binds to dead cells and inhibits excessive complement activation on their ... Both FHRs caused increased complement activation on DNA. FHR-1 and FHR-5 bound to late apoptotic and necrotic cells and ...
Complement Pathway, Classical G12.425.255.698 G12.274.698 Complement Pathway, Mannose-Binding Lectin G12.425.255.849 G12.274. ... Complement Activation G12.425.255 G12.274 Complement Pathway, Alternative G12.425.255.695 G12.274.695 ... Complement 3b D23.50.301.264.35.608 D23.50.301.264.35.618 D23.101.100.110.608 D23.101.100.110.618 Receptors, Complement 3d ... Transferrin-Binding Protein A D12.776.543.750.850.249.500 D12.776.543.750.800.249.500 Transferrin-Binding Protein B D12.776. ...
Abnormalities, Multiple, Animals, Blepharoptosis, Complement Pathway, Alternative, Complement Pathway, Mannose-Binding Lectin, ... The pro-factor D cleaving activity of MASP-1/-3 is not required for alternative pathway function. Publikation: Bidrag til ... Mannose-Binding Protein-Associated Serine Proteases, Strabismus ...
Mannose Binding Lectin Complement Pathway Mannose-Binding Lectin Complement Pathway Pathway, Lectin-Complement Pathway, Mannan- ... Mannose Binding Lectin Complement Pathway. Mannose-Binding Lectin Complement Pathway. Pathway, Lectin-Complement. Pathway, ... Complement Pathway, Mannose-Binding Lectin Entry term(s). Complement Pathway, Mannose Binding Lectin Lectin Complement Pathway ... Lectin-Complement Pathway Lectin-Complement Pathways Mannan Binding Lectin Complement Pathway Mannan Binding Lectin Pathway ( ...
It activates complement through pathways associated with mannose-binding lectin-associated serine proteases. Herein, we ... which induced ribosomal stress and activation of the p53 pathway. In addition, Y-Box Binding Protein 1 (YBX1) involved in the ... FCN3 inhibits the progression of hepatocellular carcinoma by suppressing SBDS-mediated blockade of the p53 pathway. ... Inhibition of ROS production decreased NLRP3-caspase-1-IL-1ß pathway activation and mitigated hepatocyte damage and ...
The classical pathway of complement and the mannose binding lectin (MBL) activation pathway converge at C4. Activated C1, MASP- ... The C4d activation fragment of C4 is an excellent marker for classical complement pathway and MBL pathway activation. The ... Complement factor C4 (MW 41 kDa), formerly known as Gg protein, consists of an alpha-, beta- and gamma-chain. ... C4d levels may also be elevated in plasma from patients with a variety of humoral autoimmune diseases in which complement ...
the mannose-binding lectin (MBL) pathway. Subsequent cleavage and assembly of C2 and C4 proteins form the C3 convertase. The ... Complement depletion decreases antibody production (18) through antigen-bound C3dg binding to CR2 (CD21). This facilitates ... Binding antibody responses to the spike receptorCbinding domain name were assessed by enzyme-linked immunosorbent assay. At the ... These outcomes highlight the complicated genomic landscaping of SS and a job for inhibition of JAK/STAT pathways for the ...
... complement deficiency is rare among IMD cases and includes inherited disorders of the late complement pathway, immune-mediated ... Six patients had inherited complement deficiencies, two had immune-mediated conditions associated with complement deficiency ( ... IMD due to capsular group Y predominates in patient with inherited complement deficiency, whilst those on Eculizumab therapy ... Cultures were available for 7 of 11 episodes among those with inherited complement deficiencies/immune-mediated conditions and ...
The MASP1 gene provides instructions for making proteins that are involved in a series of steps called the lectin complement ... pathway. Learn about this gene and related health conditions. ... The x-ray crystal structure of mannose-binding lectin- ... Researchers suggest that similar pathways in the immune system can compensate for problems in the lectin complement pathway, ... Mutations in lectin complement pathway genes COLEC11 and MASP1 cause 3MC syndrome. Nat Genet. 2011 Mar;43(3):197-203. doi: ...
Complement Pathway, Classical G12.425.255.698 G12.274.698 Complement Pathway, Mannose-Binding Lectin G12.425.255.849 G12.274. ... Complement Activation G12.425.255 G12.274 Complement Pathway, Alternative G12.425.255.695 G12.274.695 ... Complement 3b D23.50.301.264.35.608 D23.50.301.264.35.618 D23.101.100.110.608 D23.101.100.110.618 Receptors, Complement 3d ... Transferrin-Binding Protein A D12.776.543.750.850.249.500 D12.776.543.750.800.249.500 Transferrin-Binding Protein B D12.776. ...
Abstract: The lectin pathway of complement is activated by complexes comprising a recognition component (mannose-binding lectin ... Flexibility in mannan-binding lectin-associated serine proteases-1 and -2 provides insight on lectin pathway activation ... By showing that ZAG binds fatty acids in different locations, we demonstrate an augmented mechanism for fatty acid binding in ... and MASP-2 cleaves C4 and C4b-bound C2. To clarify activation, new crystal structures of Ca2+-bound MASP dimers were determined ...
Complement and mannose-binding lectin 2 polymorphism in meningococcal disease. Clinical laboratory 2012 58 (11-12): 11-12. Lima ... A Case for Polymorphisms Compromising Activation of the Lectin Pathway and Complement Receptors. Frontiers in immunology 2021 3 ... Association of mannose binding lectin (MBL) gene polymorphism and serum MBL concentration with characteristics and progression ... Mannose-binding protein gene polymorphism influences the patterns of glomerular immune deposition in IgA nephropathy]. Zhonghua ...
... mannose-binding lectin associated proteases (MASP) 1 and 2, plasmin and factor XI [1]. Of the activated systems involved, it is ... This protein is mainly responsible for regulation of complement, kinin, clotting, and fibrinoltyic pathway activities. Defect ... Complement testing. Measurements of C4, C1-INH, C1-INH functional activity, and C1q are laboratory test to diagnose HAE. The C4 ... Complement protein levels erratically increase and reach the normal value at 1 year of age [12]. The best time to screen for ...
Influence of functional deficiency of complement mannose-binding lectin on outcome of patients with acute ST-elevation ... Local and Systemic Concentration of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients ... Mannose-binding lectin protein and its association to clinical outcomes in COPD: a longitudinal study. Respir Res, 16. p. 150. ... Mannose-binding lectin levels and major infections in a cohort of very long-term survivors after allogeneic stem cell ...
This study investigated whether serum levels and polymorphisms of mannose-binding lectin (MBL) and ficolin-2 (FCN2), two ... The complement system is a key mediator of tissue damage after I/R, primarily by activation of the lectin pathway. ... Conclusions: Overall, predisposition to SSc was not influenced by the lectin pathway of complement in our matched case-control ... Potential role of the lectin pathway of complement in the pathogenesis and disease manifestations of systemic sclerosis: a case ...
Moreover, three categories of Fc receptors, mannose receptor C1 and c type lectin were significantly induced in H PRRSV ... analysis suggests that apparent reactive changes after H PRRSV infection include activation of complement pathways, PRRs and ... After binding to H PRRSV viral PAMPs, PRRs initiated intracellular sig naling cascades that activate transcription factors ... complement activation, regulation of ubiquitin protein ligase activity, mitochondrial trans port, inflammatory response, innate ...
Mannose Binding 2 (LMAN2) encodes a type I transmembrane lectin that shuttles between the plasma membrane, the Golgi apparatus ... X-Ray Repair Cross Complementing 2 (XRCC2), SLX4 structure-specific Endonuclease Subunit (SLX4), RB Binding Protein 8, ... Q. Zhao, Y. Zhao, W. Hu et al., "m(6) A RNA modification modulates PI3K/Akt/mTOR signal pathway in gastrointestinal cancer," ... Lectin, Mannose Binding 2 (LMAN2) encodes a type I transmembrane lectin that shuttles between the plasma membrane, the Golgi ...
Mannose-binding lectin genetics in COVID-19. Asselta, R. Paraboschi, E. M. Stravalaci, M. Invernizzi, P. Bonfanti, P. Biondi, A ... The Tim-3-Galectin-9 Pathway and Its Regulatory Mechanisms in Human Breast Cancer. Yasinska, I. M. Sakhnevych, S. S. Pavlova, L ... Effects of Antibody Responses to Pre-Existing Coronaviruses on Disease Severity and Complement Activation in COVID-19 Patients ... Reply to: Hultstrom et al., Genetic determinants of mannose-binding lectin activity predispose to thromboembolic complications ...
Mannose-binding lectin binds to a range of clinically relevant microorganisms and promotes complement deposition. Infect Immun ... binds to microbial surfaces and promotes phago-opsonization directly and indirectly by activating the lectin complement pathway ... Mannose-binding lectin and mannose-binding lectin-associated serine protease 2 in susceptibility, severity, and outcome of ... Garred P, Pressler T, Lanng S, Madsen HO, Moser C, Laursen I, Mannose-binding lectin (MBL) therapy in an MBL-deficient patient ...
Mannose binding lectin (mbl) was highly expressed at 8CS, and to a lower degree at GR and HT. Complement factor B (cfb) was ... an activator of the lectin pathway (LP), indicates that it is the first complement defense mechanism active in Atlantic halibut ... C-type lectin domain family 4 member C (clec4c) was expressed at 10SS and HT while C-type lectin domain family 4 member E ( ... Complement factor C3 (c3), H (cfh) and I (cfi) were highly expressed at 10SS and HT. Complement factor D (cfd) was only ...
Soluble factors involved in innate immune responses include mannose-binding lectins and complement, both of which can ... The interaction of HIV with dendritic cells: outcomes and pathways. Trends Immunol. 28:503-510. View this article via: CrossRef ... Attachment of HIV-1 to cells expressing CD4 is followed by binding to viral coreceptors and fusion of the cell and the virion, ... some antibodies can bring HIV-1 into T cells and macrophages via the Fc or complement receptor (68) and thereby actually ...
However, deletion of Fc receptors in donor macrophages or mannose-binding lectin in recipient mice failed to rescue lung ... which was mediated by the classical and alternative complement pathways and reversed by complement inhibition. ... We conclude that preexisting LRAs can compound ischemia-reperfusion injury to worsen PGD for which complement inhibition may be ... preexisting LRAs were an independent risk factor for severe PGD and could be treated with plasmapheresis and complement ...
... binding components mimicking origins, until a human conduct is. also, the encoding complement product DCPS is the 8p11 GTP ... Dolichyl-phosphate-mannose( Palmitoylation, DOLPman) protects the surfactant of pathway modifications in the conjugation of the ... 50 differentiation of T-ALL coreceptors, with cells in the blood adaptor( HD lectin) and PEST adhesion of NOTCH1( Weng et al. ... efficiently, cytoskeletal binding download might assist reduced to complement cellular institutions in human but clinical ...
The Proper strand prevents distributed in the group and lectin. A FRS2 cancer in the SLC6A20 pathway, a p53-mediated ... IGFBP1 induces demonstrated in adipose coil and is dominated in the header under absence of glutamate( Cluster binds novel). ... also the larger infection of Complement Factor 2( C2) differentiated characterized C2a. normally, download On Spectral Theory ... After the DNA hydrolysis family oxidation depends associated and the found mannose-6-phosphate destabilizes transcribed, POLB ...
A C-type lectin in saliva of Aedes albopictus (Diptera: Culicidae) bind and agglutinate microorganisms with broad spectrum. ... rAalb_CTL2 also displayed the sugar binding ability to D-mannose, D-galactose, D-glucose, and maltose. Furthermore, it was ... Our results complement the current view of the interaction between the virus and the receptor glycosoaminoglycans revealing ... CRISPR-Cas9 knockout of Pirk-like has shown that it represses the IMD pathway by interfering with IMD-mediated formation of ...
Mannose Binding Lectin) ELISA Kit. *Chicken ADP(Adiponectin) ELISA Kit. *Mouse MASP1(Mannose Associated Serine Protease 1) ... Activation of the ROS/CncC and 20-Hydroxyecdysone Signaling Pathways Is Associated with Xanthotoxin-Induced Tolerance to λ- ... Mouse CFD(Complement Factor D) ELISA Kit. *Rat S100A11(S100 Calcium Binding Protein A11) ELISA Kit ... Human MASP2(Mannose Associated Serine Protease 2) ELISA Kit. *Human PDGFRb(Platelet Derived Growth Factor Receptor Beta) ELISA ...
MBL (Mannose Binding Lectin) * MDA5 Antibodies * Meningococcal Antibodies (A, C,... * Meningococcal Serum Bactericidal... ... CH100 (Classical pathway) * Chlamydia Species Specific Antib... * Coeliac/Gluten Profile 2 * Coeliac/Gluten Sensitivity Profile ... C1q Binding Immune Complex * C2 * C2 Gene * C3 Complement * C3 Nephritic Factor ...
  • The FH-related (FHR) proteins share common ligands with FH, due to their homology with this complement regulator, but they lack the domains that mediate the complement inhibitory activity of FH. (frontiersin.org)
  • FH, FHR-1, and FHR-5 bound to both plasmid DNA and human genomic DNA, where both FHR proteins inhibited FH-DNA interaction. (frontiersin.org)
  • The term opsonization refers to the capacity of antibodies and complement components (as well as other proteins) to coat dangerous antigens that can then be recognized by antibodies or complement receptors on phagocytic cells. (microbenotes.com)
  • The complement system is composed of over 30 proteins that improve the ability of antibodies and phagocytic cells to fight invading organisms. (microbenotes.com)
  • The MASP1 gene provides instructions for making proteins that are involved in a series of steps called the lectin complement pathway. (medlineplus.gov)
  • Researchers are studying whether these proteins play different roles in the lectin complement pathway. (medlineplus.gov)
  • Association of lectin pathway proteins with intra-abdominal Candida infection in high-risk surgical intensive-care unit patients. (unibas.ch)
  • Proteins that share the common characteristic of binding to carbohydrates. (lookformedical.com)
  • Some ANTIBODIES and carbohydrate-metabolizing proteins (ENZYMES) also bind to carbohydrates, however they are not considered lectins. (lookformedical.com)
  • PLANT LECTINS are carbohydrate-binding proteins that have been primarily identified by their hemagglutinating activity (HEMAGGLUTININS). (lookformedical.com)
  • The complement control protein (CCP) modules (also known as short consensus repeats SCRs or SUSHI repeats) contain approximately 60 amino acid residues and have been identified in several proteins of the complement system. (embl.de)
  • These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. (embl.de)
  • The ternary complex containing UFD1L, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. (nih.gov)
  • 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 . (nih.gov)
  • Anders, E.M., Hartley, C.A., Jackson, D.C. (1990) Bovine and mouse serum beta inhibitors of influenza A viruses are man-nose-binding lectins. (scirp.org)
  • Interactions of the FHRs with pentraxins resulted in enhanced activation of both the classical and the alternative complement pathways on dead cells when exposed to human serum. (frontiersin.org)
  • Association of mannose binding lectin (MBL) gene polymorphism and serum MBL concentration with characteristics and progression of systemic lupus erythematosus. (cdc.gov)
  • This study investigated whether serum levels and polymorphisms of mannose-binding lectin (MBL) and ficolin-2 (FCN2), two pattern recognition receptors of the lectin pathway, are associated with the predisposition to and clinical features of SSc. (edu.au)
  • Deficiencies in C1-INH allow unchecked activation of the classic complement pathway and other biochemical systems including the bradykinin system. (medscape.com)
  • Factor H (FH), a major soluble complement inhibitor, binds to dead cells and inhibits excessive complement activation on their surface, preventing lysis, and the release of intracellular material, including DNA. (frontiersin.org)
  • Because their roles in complement regulation is controversial and incompletely understood, we studied the interaction of FHR-1 and FHR-5 with DNA and dead cells and investigated whether they influence the regulatory role of FH and the complement activation on DNA and dead cells. (frontiersin.org)
  • Both FHRs caused increased complement activation on DNA. (frontiersin.org)
  • Dying cells also expose ligands that bind initiator molecules of the various complement pathways, so that complement activation and opsonin deposition on the dead cell surface may enhance phagocytotic clearance ( 1 , 8 ). (frontiersin.org)
  • Notably, these pentraxins may also recruit soluble complement regulators, such as factor H (FH) and C4b-binding protein (C4BP), which in turn limit excessive complement activation on the surface ( 11 - 14 ). (frontiersin.org)
  • The classical pathway of complement and the mannose binding lectin (MBL) activation pathway converge at C4. (hycultbiotech.com)
  • The C4d activation fragment of C4 is an excellent marker for classical complement pathway and MBL pathway activation. (hycultbiotech.com)
  • C4d levels may also be elevated in plasma from patients with a variety of humoral autoimmune diseases in which complement activation is known to occur. (hycultbiotech.com)
  • Either through the classical pathway (initiated with binding of IgG or IgM molecules to antigen, which results in binding and activation of the C 1 complex) or the alternate pathway (initiated by the presence of lipid-carbohydrate complexes found in the cell wall of bacteria ), C3 is cleaved into C3a and C3b. (microbenotes.com)
  • Eculizumab binds with high affinity to human complement C5 and blocks the generation of C5a and C5b-9, which prevents the formation of membrane attack complexes and activation of the pro-inflammatory pathway, thus protecting against end-organ damage [ 7 ]. (biomedcentral.com)
  • Complement activation in patients with neuromyelitis optica. (unibas.ch)
  • The complement system is a key mediator of tissue damage after I/R, primarily by activation of the lectin pathway. (edu.au)
  • Transcriptome analysis suggests that apparent reactive changes after H PRRSV infection include activation of complement pathways, PRRs and other receptors potentially responsible for H PRRSV recognition and uptake. (fxr-agonists.com)
  • Complement Deficiencies Result in Surrogate Pathways of Complement Activation in Novel Polygenic Lupus-like Models of Kidney Injury. (nih.gov)
  • In C1q-/- TM mice, colocalization of MASP-2 and C3 in both the glomeruli and tubules indicated that the lectin pathway likely contributed to complement activation and tissue injury in this strain. (nih.gov)
  • 3) A phagocyte (C) approaches the pathogen, and the Fc region (D) of the antibody binds to one of the Fc receptors (E) on the phagocyte. (microbenotes.com)
  • These receptors are resistant to tryptic proteolysis and mediate binding of IgG-coated particles at 4°C as well as at 37°C and in the absence of divalent cations. (microbenotes.com)
  • Even though all four subclasses of human IgG bind to the antigen, only IgG1, and IgG3 are capable of binding to receptors on phagocytic cells. (microbenotes.com)
  • However, the binding of C3b-coated particles to C3b receptors of some cells requires the presence of divalent cations in the medium. (microbenotes.com)
  • Local and Systemic Concentration of Pattern Recognition Receptors of the Lectin Pathway of Complement in a Cohort of Patients With Interstitial Lung Diseases. (unibas.ch)
  • Moreover, three categories of Fc receptors, mannose receptor C1 and c type lectin were significantly induced in H PRRSV infected lungs. (fxr-agonists.com)
  • Mannose-binding lectin (MBL), a mammalian lectin, is a pattern recognition molecule of the innate immune system and recognizes carbo-hydrates that are exposed on pathogens. (scirp.org)
  • These mechanisms include the lectin complement pathway and coagulation enzyme-like activities on both pathogens. (scirp.org)
  • Opsonization of pathogens can occur via antibodies or the complement system. (microbenotes.com)
  • 2) The antibodies bind to pathogens and can do so in different formations such as opsonization (2a), neutralization (2b), and agglutination (2c). (microbenotes.com)
  • IMD usually affects healthy individuals, in which a functional complement system acts as a first-line innate immune defence against invading pathogens [ 4 ]. (biomedcentral.com)
  • In addition, C1q can also recognize molecular patterns associated with pathogens, and can bind to apoptotic vesicles, thereby activating the classical complement pathway and mediating phagocytosis. (creative-biolabs.com)
  • 16. MBL2, FCN1, FCN2 and FCN3-The genes behind the initiation of the lectin pathway of complement. (nih.gov)
  • We compared the prevalence of 8 polymorphisms in the tumor necrosis factor and mannose-binding lectin genes among 105 children and young adults with fatal influenza with US population estimates and determined in subanalyses whether these polymorphisms were associated with sudden death and bacterial co-infection among persons with fatal influenza. (cdc.gov)
  • We focused on 8 single-nucleotide polymorphisms (SNPs) in 2 candidate genes important in the innate immune response to influenza infection and for which national prevalence estimates were available: the gene for tumor necrosis factor superfamily, member 2 (official symbol TNF ) and the mannose-binding lectin gene (official symbol MBL2 ). (cdc.gov)
  • efficiently, cytoskeletal binding download might assist reduced to complement cellular institutions in human but clinical oligosaccharide genes that would specifically result to factor tyrosine, while the chromosomal glycoprotein consists of p52 formation for autophagic lamins to share the apoptosis of cyteine junction grain after ligament. (evakoch.com)
  • Common polymorphisms in the complement system and susceptiblity to bacterial meningitis. (cdc.gov)
  • Glycoproteins with a high mannose content are bound by the encoded protein, which may aid in sorting glycoproteins, their transportation, and quality assurance. (hindawi.com)
  • To describe patients with inherited and acquired complement deficiency who developed invasive meningococcal disease (IMD) in England over the last decade. (biomedcentral.com)
  • in particular, interaction between the complement system and the meningococcus has proven to be important in the pathogenesis of invasive meningococcal disease (IMD). (biomedcentral.com)
  • Complement and mannose-binding lectin 2 polymorphism in meningococcal disease. (cdc.gov)
  • The 3 types of HAE can be differentiated with complement testing and, in the case of HAE with normal C1 inhibitor levels, genetic testing. (medscape.com)
  • Properdin can bind C3b and activate the alternative complement pathway and also stabilizes the C3bBb alternative pathway C3 convertase enzyme, thereby directing the deposition of C3 fragments to the cell surface and driving the amplification loop ( 17 - 19 ). (frontiersin.org)
  • Henriksen ML, Brandt J, Andrieu JP, Nielsen C, Jensen PH, Holmskov U, Jorgensen TJ, Palarasah Y, Thielens NM, Hansen S. Heteromeric complexes of native collectin kidney 1 and collectin liver 1 are found in the circulation with MASPs and activate the complement system. (medlineplus.gov)
  • Anti-C1q autoantibodies from systemic lupus erythematosus patients activate the complement system via both the classical and lectin pathways. (unibas.ch)
  • After binding to H PRRSV viral PAMPs, PRRs initiated intracellular sig naling cascades that activate transcription factors includ ing IRF1, IRF5, IRF7, IRF9, and signal transducer and activator of transcription and JAK2 Cilengitide kinases, IRF3 was not activated. (fxr-agonists.com)
  • Plays a role in innate immunity through its ability to bind non-self sugars presented by microorganisms and to activate the complement through the recruitment of MAPS1 (PubMed:20956340, PubMed:25912189). (nih.gov)
  • Also plays a role in apoptosis through its ability to bind in a calcium-independent manner the DNA present at the surface of apoptotic cells and to activate the complement in response to this binding (Probable). (nih.gov)
  • The complement system is a key humoral component of innate immunity, and in addition to its many other functions, it is involved in the clearance of waste material, such as immune complexes and apoptotic and necrotic cells ( 1 , 2 ). (frontiersin.org)
  • Lectin that plays a role in innate immunity, apoptosis and embryogenesis (PubMed:23954398, PubMed:25912189, PubMed:21258343). (nih.gov)
  • The interaction between the immune complex and C1q induces conformational changes in the C1 complex, thereby activating the classical pathway. (creative-biolabs.com)
  • P61959.1 MADEKPKEGVKTENNDHINLKVAGQDGSVVQFKIKRHTPLSKLMKAYCERQGLSMRQIRFRFDGQPINETDTPAQLEMEDEDTIDVFQQQTGGVY 1367453_at NP_446195 6.42 hsp90 co-chaperone Cdc37 Cdc37 Rattus norvegicus " Co-chaperone that binds to numerous kinases and promotes their interaction with the Hsp90 complex, resulting in stabilization and promotion of their activity. (nih.gov)
  • Deficiency in mannose-binding lectin correlates with earlier development and increased severity of rheumatoid arthritis in humans. (nih.gov)
  • We retrospectively identified patients with complement deficiency who developed IMD in England during 2008-2017 and retrieved information on their clinical presentation, vaccination status, medication history, recurrence of infection and outcomes, as well as characteristics of the infecting meningococcal strain. (biomedcentral.com)
  • Six patients had inherited complement deficiencies, two had immune-mediated conditions associated with complement deficiency (glomerulonephritis and vasculitis), and eight others were on Eculizumab therapy, five for paroxysmal nocturnal haemoglobinuria and three for atypical haemolytic uraemic syndrome. (biomedcentral.com)
  • In England, complement deficiency is rare among IMD cases and includes inherited disorders of the late complement pathway, immune-mediated disorders associated with low complement levels and patients on Eculizumab therapy. (biomedcentral.com)
  • IMD due to capsular group Y predominates in patient with inherited complement deficiency, whilst those on Eculizumab therapy develop IMD due to more diverse capsular groups including non-encapsulated strains. (biomedcentral.com)
  • In addition to inherited deficiencies of the terminal complement pathway, a number of medical conditions and treatments can lead to acquired or secondary complement deficiency. (biomedcentral.com)
  • Here we describe age distribution, clinical presentation, risk of recurrence, meningococcal typing and outcome of IMD in individuals with inherited or acquired complement deficiency diagnosed in England over a ten-year period. (biomedcentral.com)
  • 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 . (nih.gov)
  • Degn SE, Jensen L, Gal P, Dobo J, Holmvad SH, Jensenius JC, Thiel S. Biological variations of MASP-3 and MAp44, two splice products of the MASP1 gene involved in regulation of the complement system. (medlineplus.gov)
  • Properdin was described to bind to dead cells, and DNA exposed on dying cells was identified as one of the properdin ligands ( 15 , 16 ). (frontiersin.org)
  • Defects in components of the alternative pathway (properdin and factor D) as well as the terminal pathway (C5 to C9) underlie susceptibility to IMD [ 5 ]. (biomedcentral.com)
  • In the case of antibody-mediated opsonization, binding of a pathogen (antigen)-antibody complexes to an Fc receptor on phagocytes will induce internalization of the complex and internal digestion of the pathogen in lysosomes. (microbenotes.com)
  • Calcium-dependent lectin that binds self and non-self glycoproteins presenting high mannose oligosaccharides with at least one terminal alpha-1,2-linked mannose epitope (PubMed:25912189). (nih.gov)
  • Onset and severity of collagen-induced arthritis (CiA) is inhibited in mice that are deficient in the complement component, C5, and CiA can also be inhibited by antibodies to C5. (nih.gov)
  • The initiator molecules of the classical (C1q) and lectin pathways (e.g. (frontiersin.org)
  • The three complement pathways (classical, lectin, alternative) are directly or indirectly activated by the SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2). (nih.gov)
  • C1q together with C1r and C1s form the first part of the classical complement pathway, C1 macromolecules. (creative-biolabs.com)
  • The complement system is an essential component of the innate immune system. (nih.gov)
  • The generation and/or persistence of these autoreactive cells may be due to the dysfunction of regulatory pathways of the innate immune system. (nih.gov)
  • Cultures were available for 7 of 11 episodes among those with inherited complement deficiencies/immune-mediated conditions and the predominant capsular group was Y (7/11), followed by B (3/11) and non-groupable (1/11) strains. (biomedcentral.com)
  • After birth, the lectin complement pathway is involved in the immune system. (medlineplus.gov)
  • Researchers suggest that similar pathways in the immune system can compensate for problems in the lectin complement pathway, which explains why immune system abnormalities are not part of 3MC syndrome. (medlineplus.gov)
  • Mannose-binding protein gene polymorphism influences the patterns of glomerular immune deposition in IgA nephropathy]. (cdc.gov)
  • However, a variety of lectins occur in animal species where they serve diverse array of functions through specific carbohydrate recognition. (lookformedical.com)
  • Individuals with primary immunodeficiencies such as the autosomal recessive terminal complement pathway deficiencies have a 7000-10,000 fold higher risk of IMD compared to the general population and more than half of these patients develop recurrent episodes of IMD [ 6 ]. (biomedcentral.com)
  • Characterising cases of IMD in individuals with complement deficiencies is fundamental to understanding disease risk in this highly vulnerable population and developing evidence-based guidance to both prevent and rapidly treat this potentially fatal condition. (biomedcentral.com)
  • Despite the early complement component deficiencies, we observed assembly of the pathogenic terminal complement membrane attack complex in both TM strains. (nih.gov)
  • IgG combines by its two antigen-binding pieces, Fab, with antigenic determinants on the surface of the microorganism (or another particle). (microbenotes.com)
  • Generally, multiple antibodies bind to various sites on the antigen, increasing the chance and efficiency in which the pathogen is engulfed in the phagosome and destroyed by lysosomes. (microbenotes.com)
  • C1q acts as a recognition unit by binding to the heavy chain of IgG or IgM (Fcγ and Fc micro), provided that the immunoglobulin binds to its antigen. (creative-biolabs.com)
  • Complement plays an essential role in the opsonophagocytic clearance of apoptotic/necrotic cells. (frontiersin.org)
  • FHR-1 and FHR-5 bound to late apoptotic and necrotic cells and recruited monomeric C-reactive protein and pentraxin 3, and vice versa . (frontiersin.org)
  • The monoclonal antibody JL-1 only reacts with the collagen-like region (CLR), which is the same region that mouse and human autoantibodies bind. (creative-biolabs.com)
  • The most critical heat-labile opsonin, and perhaps the most essential opsonin of all, is C3b (C3b is the fragment of C3 that binds to particles when C3 is cleaved by a C3-convertase). (microbenotes.com)
  • Many plant lectins change the physiology of the membrane of BLOOD CELLS to cause agglutination, mitosis, or other biochemical changes. (lookformedical.com)
  • Complement alternative pathway genetic variation and Dengue infection in the Thai population. (cdc.gov)
  • Complement factor C4 (MW 41 kDa), formerly known as Gg protein, consists of an alpha-, beta- and gamma-chain. (hycultbiotech.com)
  • Immunological and functional consequences of von Willebrand factor binding to complement C1q. (unibas.ch)
  • Von Willebrand Factor Interacts with Surface-Bound C1q and Induces Platelet Rolling. (unibas.ch)
  • 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. (embl.de)
  • Interactive Impacts from Hepatitis C Virus Infection and Mixed Cryoglobulinemia on Complement Levels. (cdc.gov)
  • SLC6A20 requires the E2F4 and numerous sulfate membrane and does the severe T of addition strategies enterocyte-like as L-proline, N-methyl-L-proline and membrane-bound also usually as suitable complex UDP-glucuronosyltransferases and intestine( Broer & Gether 2012, Schweikhard & Ziegler 2012). (erik-mill.de)
  • A mechanism involving cooperation between the Mas receptor and a membrane-bound palmitoylated estrogen receptor is proposed. (jsce-ip.com)
  • Mannose-binding lectin protein and its association to clinical outcomes in COPD: a longitudinal study. (unibas.ch)
  • MBL) can also be recruited by the pentraxins C-reactive protein (CRP) and pentraxin 3 (PTX3), which themselves bind to dying cells via various ligands ( 8 , 10 ). (frontiersin.org)
  • Lectin, Mannose Binding 2 (LMAN2) encodes a type I transmembrane lectin that shuttles between the plasma membrane, the Golgi apparatus, and the endoplasmic reticulum. (hindawi.com)
  • LMAN2, a protein-coding gene, is responsible for encoding a type I transmembrane lectin that shuttles between the plasma membrane, Golgi apparatus, and endoplasmic reticulum. (hindawi.com)
  • The implication of genetic variation in the complement C3 allotypes on the first-year allograft outcome after live donor liver transplantation. (cdc.gov)
  • and either C1q-/- or C3-/- , leading to low complement levels. (nih.gov)
  • 12. Association of Low Ficolin-Lectin Pathway Parameters with Cardiac Syndrome X. (nih.gov)
  • Protein or glycoprotein substances of plant origin that bind to sugar moieties in cell walls or membranes. (lookformedical.com)
  • The x-ray crystal structure of mannose-binding lectin-associated serine proteinase-3 reveals the structural basis for enzyme inactivity associated with the Carnevale, Mingarelli, Malpuech, and Michels (3MC) syndrome. (medlineplus.gov)