Complement Pathway, Mannose-Binding Lectin
Complement Pathway, Alternative
Complement Pathway, Classical
Complement System Proteins
Genetic Predisposition to Disease
Mannose-Binding Protein-Associated Serine Proteases
Complement Factor B
Complement Inactivator Proteins
Complement Factor D
Complement C3-C5 Convertases
Complement Factor H
Complement Membrane Attack Complex
Complement C3b Inactivator Proteins
Complement Activating Enzymes
Complement Inactivating Agents
Complement Hemolytic Activity Assay
Molecular Sequence Data
Complement C1 Inactivator Proteins
Blood Bactericidal Activity
Receptors, Complement 3b
Complement Fixation Tests
Complement Factor I
Complement C4b-Binding Protein
Receptor, IGF Type 2
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.
Mannose-binding protein-associated serine protease
List of MeSH codes (G04)
Malpuech facial clefting syndrome
Innate immune system
Mannan-binding lectin-associated serine protease-2
Outline of immunology
Pattern recognition receptor
Complement component 1s
List of EC numbers (EC 3)
Dietary sugars inhibit biologic functions of the pattern recognition molecule, mannose-binding lectin
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- 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)
PROTEIN-ASSOCIATED SERINE PROTEASES1
- 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)
Inhibited by antibodies1
- 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)
- 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)