Complement C3
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.
Complement C4
Complement C4a
Complement C3a
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.
Complement C1q
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.
Complement C5a
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.
Complement Activation
Complement C4b
Complement C5
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.
Complement C3b
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.
Complement System Proteins
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).
Complement C6
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.
Complement C3c
Complement C3d
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).
Complement C2
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).
Complement C9
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.
Receptors, Complement
Complement C1s
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).
Complement Membrane Attack Complex
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.
Complement C1r
A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.
Complement Inactivator Proteins
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.
Complement C7
A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.
Complement C3-C5 Convertases
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.
Complement Factor B
Complement Pathway, Alternative
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 Pathway, Classical
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.
Complement C8
A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.
Complement C1
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.
Receptors, Complement 3b
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.
Complement Factor H
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).
Complement C5b
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.
Complement C2a
Receptor, Anaphylatoxin C5a
Complement Activating Enzymes
Complement Inactivating Agents
Complement Hemolytic Activity Assay
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.
Complement C1 Inactivator Proteins
Receptors, Complement 3d
Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.
Anaphylatoxins
Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.
Complement Fixation Tests
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.
Complement Factor D
Complement Factor I
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.
Complement C4b-Binding Protein
Complement C3b Inactivator Proteins
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.
Antigens, CD55
Complement C3-C5 Convertases, Classical Pathway
Complement C2b
Antigens, CD59
Cobra Venoms
Antigen-Antibody Complex
Steroid 21-Hydroxylase
An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).
Complement C3-C5 Convertases, Alternative Pathway
Complement C1 Inhibitor Protein
An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.
Immunoglobulin G
Hemolysis
Complement C3 Convertase, Alternative Pathway
Complement C5 Convertase, Classical Pathway
Molecular Sequence Data
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.
Complement C3 Convertase, Classical Pathway
Antigens, CD46
Opsonin Proteins
Blood Proteins
Lupus Erythematosus, Systemic
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
Complement C5 Convertase, Alternative Pathway
Phagocytosis
Amino Acid Sequence
Complement Pathway, Mannose-Binding Lectin
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.
Properdin
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.
Complement C5a, des-Arginine
Macrophage-1 Antigen
Protein Binding
Neutrophils
Base Sequence
Kidney Glomerulus
Serum
Glomerulonephritis, Membranoproliferative
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.
Immunoglobulin M
Schistosoma
Genetic Complementation Test
Enzyme-Linked Immunosorbent Assay
An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.
Mice, Knockout
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Glomerulonephritis
Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.
Arteriolosclerosis
Major Histocompatibility Complex
The genetic region which contains the loci of genes which determine the structure of the serologically defined (SD) and lymphocyte-defined (LD) TRANSPLANTATION ANTIGENS, genes which control the structure of the IMMUNE RESPONSE-ASSOCIATED ANTIGENS, HUMAN; the IMMUNE RESPONSE GENES which control the ability of an animal to respond immunologically to antigenic stimuli, and genes which determine the structure and/or level of the first four components of complement.
Erythrocytes
Autoantibodies
Cells, Cultured
RNA, Messenger
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Macrophages
The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)
Immunity, Innate
Peptide Fragments
Mutation
Rabbits
Disease Models, Animal
Cloning, Molecular
Binding Sites
Blood Bactericidal Activity
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.
Antigens, CD
Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.
Electrophoresis, Polyacrylamide Gel
Mannose-Binding Lectin
Alleles
Antibodies
Complement C3 Nephritic Factor
An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.
Glycoproteins
Immunoglobulins
Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.
Haptoglobins
DNA
A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).
Surface Plasmon Resonance
A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.
Peptides, Cyclic
Lupus Nephritis
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Antibodies, Antinuclear
Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.
Sequence Homology, Amino Acid
Blotting, Western
Cosmids
Polymerase Chain Reaction
In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.
Gene Expression Regulation
Biological Markers
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
Inflammation
Carrier Proteins
Mannose-Binding Protein-Associated Serine Proteases
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.
Adrenal Hyperplasia, Congenital
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.
Species Specificity
The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.
Kidney
Phenotype
Immunologic Factors
Protein Structure, Tertiary
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
Immunohistochemistry
Gene Dosage
The number of copies of a given gene present in the cell of an organism. An increase in gene dosage (by GENE DUPLICATION for example) can result in higher levels of gene product formation. GENE DOSAGE COMPENSATION mechanisms result in adjustments to the level GENE EXPRESSION when there are changes or differences in gene dosage.
Haplotypes
Membrane Proteins
HLA Antigens
Sequence Homology, Nucleic Acid
Gene Expression
Monocytes
Fibrinogen
Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products.
Exons
B-Lymphocytes
Flow Cytometry
Technique using an instrument system for making, processing, and displaying one or more measurements on individual cells obtained from a cell suspension. Cells are usually stained with one or more fluorescent dyes specific to cell components of interest, e.g., DNA, and fluorescence of each cell is measured as it rapidly transverses the excitation beam (laser or mercury arc lamp). Fluorescence provides a quantitative measure of various biochemical and biophysical properties of the cell, as well as a basis for cell sorting. Other measurable optical parameters include light absorption and light scattering, the latter being applicable to the measurement of cell size, shape, density, granularity, and stain uptake.
Antibody Formation
Serine Endopeptidases
Streptococcus pneumoniae
Collectins
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.
Restriction Mapping
Genes
DNA Primers
C-Reactive Protein
Genotype
Up-Regulation
Lipopolysaccharides
Lipid-containing polysaccharides which are endotoxins and important group-specific antigens. They are often derived from the cell wall of gram-negative bacteria and induce immunoglobulin secretion. The lipopolysaccharide molecule consists of three parts: LIPID A, core polysaccharide, and O-specific chains (O ANTIGENS). When derived from Escherichia coli, lipopolysaccharides serve as polyclonal B-cell mitogens commonly used in laboratory immunology. (From Dorland, 28th ed)
Steroid Hydroxylases
Blotting, Northern
T-Lymphocytes
Lymphocytes responsible for cell-mediated immunity. Two types have been identified - cytotoxic (T-LYMPHOCYTES, CYTOTOXIC) and helper T-lymphocytes (T-LYMPHOCYTES, HELPER-INDUCER). They are formed when lymphocytes circulate through the THYMUS GLAND and differentiate to thymocytes. When exposed to an antigen, they divide rapidly and produce large numbers of new T cells sensitized to that antigen.
DNA, Complementary
Blotting, Southern
Cytokines
Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.
Macular Degeneration
Disease Susceptibility
Models, Molecular
Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
A mass spectrometric technique that is used for the analysis of large biomolecules. Analyte molecules are embedded in an excess matrix of small organic molecules that show a high resonant absorption at the laser wavelength used. The matrix absorbs the laser energy, thus inducing a soft disintegration of the sample-matrix mixture into free (gas phase) matrix and analyte molecules and molecular ions. In general, only molecular ions of the analyte molecules are produced, and almost no fragmentation occurs. This makes the method well suited for molecular weight determinations and mixture analysis.
Reverse Transcriptase Polymerase Chain Reaction
Cell Membrane
Pedigree
Case-Control Studies
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
Polymorphism, Restriction Fragment Length
Gene Frequency
Guinea Pigs
Immune Adherence Reaction
A method for the detection of very small quantities of antibody in which the antigen-antibody-complement complex adheres to indicator cells, usually primate erythrocytes or nonprimate blood platelets. The reaction is dependent on the number of bound C3 molecules on the C3b receptor sites of the indicator cell.
Escherichia coli
A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.
Immunoelectrophoresis
A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.
Staphylococcus aureus
Transfection
Liver
Lung
Arthritis, Rheumatoid
A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.
Fluorescent Antibody Technique
Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy.
Interleukin-6
Protein Conformation
The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).
Epithelial Cells
Cells that line the inner and outer surfaces of the body by forming cellular layers (EPITHELIUM) or masses. Epithelial cells lining the SKIN; the MOUTH; the NOSE; and the ANAL CANAL derive from ectoderm; those lining the RESPIRATORY SYSTEM and the DIGESTIVE SYSTEM derive from endoderm; others (CARDIOVASCULAR SYSTEM and LYMPHATIC SYSTEM) derive from mesoderm. Epithelial cells can be classified mainly by cell shape and function into squamous, glandular and transitional epithelial cells.
Structure-Activity Relationship
Gene Library
Signal Transduction
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
Genetic Predisposition to Disease
Hemoglobinuria, Paroxysmal
A condition characterized by the recurrence of HEMOGLOBINURIA caused by intravascular HEMOLYSIS. In cases occurring upon cold exposure (paroxysmal cold hemoglobinuria), usually after infections, there is a circulating antibody which is also a cold hemolysin. In cases occurring during or after sleep (paroxysmal nocturnal hemoglobinuria), the clonal hematopoietic stem cells exhibit a global deficiency of cell membrane proteins.
Polymorphism, Single Nucleotide
Immune Complex Diseases
Group of diseases mediated by the deposition of large soluble complexes of antigen and antibody with resultant damage to tissue. Besides SERUM SICKNESS and the ARTHUS REACTION, evidence supports a pathogenic role for immune complexes in many other IMMUNE SYSTEM DISEASES including GLOMERULONEPHRITIS, systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC) and POLYARTERITIS NODOSA.
Complement activation and expression of membrane regulators in the middle ear mucosa in otitis media with effusion. (1/225)
The aetiopathogenesis of chronic otitis media with effusion (OME) in children is not yet fully understood. OME is characterized by metaplasia of the epithelium and accumulation of sticky, glue-like effusion in the middle ear containing different mediators of inflammation, including activation fragments of the complement system. Here we examined whether the fluid phase complement activation is reflected in the middle ear mucosa and how the mucosa is protected against the cytolytic activity of complement. Mucosal biopsies from 18 middle ears of children with a history of chronic OME were taken. The biopsies were analysed by immunofluorescence microscopy after staining for complement fragments iC3b/C3c, C3d and C9, and regulators membrane cofactor protein (MCP; CD46), decay-accelerating factor (DAF; CD55) and protectin (CD59). There was a strong staining for iC3b/C3c, and a weaker one for C3d and C9 on the surface of the middle ear epithelial cells of OME patients but not in controls without OME. MCP was expressed on the hyperplastic three to four outer cell layers of the epithelium, while CD59 was expressed throughout the middle ear mucosa. The results suggest a strong ongoing complement activation and consequent inflammation in the middle ear cavity. Unrestricted complement damage of the epithelial lining is prevented by the strong expression of MCP and CD59. (+info)Function of the factor I modules (FIMS) of human complement component C6. (2/225)
In order to elucidate the function of complement component C6, truncated C6 molecules were expressed recombinantly. These were either deleted of the factor I modules (FIMs) (C6des-748-913) or both complement control protein (CCP) modules and FIMs (C6des-611-913). C6des-748-913 exhibited approximately 60-70% of the hemolytic activity of full-length C6 when assayed for Alternative Pathway activity, but when measured for the Classical Pathway, C6des-748-914 was only 4-6% as effective as C6. The activity difference between C6 and C6des-748-913 for the two complement pathways can be explained by a greater stability of newly formed metastable C5b* when produced by the Alternative Pathway compared with that made by the Classical Pathway. The half-lives of metastable C5b* and the decay of (125)I-C5b measured from cells used to activate the Alternative Pathway were found to be about 5-12-fold longer than those same parameters derived from cells that had activated the Classical Pathway. (125)I-C5 binds reversibly to C6 in an ionic strength-dependent fashion, but (125)I-C5 binds only weakly to C6des-FIMs and not at all to C6des-CCP/FIMs. Therefore, although the FIMs are not required absolutely for C6 activity, these modules promote interaction of C6 with C5 enabling a more efficient bimolecular coupling ultimately leading to the formation of the C5b-6 complex. (+info)On the mechanism of cytolysis by complement: evidence on insertion of C5b and C7 subunits of the C5b,6,7 complex into phospholipid bilayers of erythrocyte membranes. (3/225)
The doughnut hypothesis of cytolysis by complement [Mayer, M. M. (1972) Proc. Nat. Acad. Sci. USA 69, 2954-2958] describes an annular structure made up of C5b-9 (complement factors C5b, C6, C7, C8, and C9) which becomes inserted in the lipid bilayer of the cell membrane, thus creating a hole. We now present initial explorations of this hypothesis. EAC1-6 and EAC1-7 (sheep erythrocytes carrying rabbit antibody and complement factors C1 through C6 or C1 through C7, respectively), prepared with either 125I-C3 or 125I-C5 were incubated with trypsin and the release of bound 125I was measured. In the case of 125I-C3, all of the radioactivity was released by trypsin from both intermediates. With 125I-C5, trypsin released all of the 125I from EAC1-6, but only 40-55% from EAC1-7. Possible reasons for resistance of the C5b subunit in EAC1-7 to tryptic digestion are discussed; in terms of the doughnut hypothesis it would be due to shielding by lipid molecules as a consequence of insertion into the lipid bilayer. In accord with this interpretation we have also found that C5b in EAC1-7, but not in EAC1-6, resists elution by 0.3 M NaC1. Similarly, we have found that 125I-C7 in EAC1-7 resists stripping by trypsin. Hence, we now propose the hypothesis that hydrophobic polypeptide chains from the C5b and the C7 subunits of C5b,6,7 complex become inserted in the phospholipid bilayer and that subsequent reactions with C8 and C9 open a channel across the membrane. (+info)Increased ion permeability of planar lipid bilayer membranes after treatment with the C5b-9 cytolytic attack mechanism of complement. (4/225)
The ion permeability of planar lipid bilayers, as measured electrically, was found to increase modestly upon treatment with purified complement complex C5b,6 and complement components C7 and C8. The subsequent addition C9 greatly amplified this change. No permeability changes occurred when components were added individually to the membrane, or when they were used in paired combinations, or when C5b, C7, C8, and C9 were admixed prior to addition. Thus, there is a significant parallel between the permeability changes induced in the model membrane and damage produced in biological membranes by the C5b-9 complement attack sequence. The efficiency of membrane action by C5b-9 was critically dependent on the order in whcih components were added to the membrane. There were also differences in the electrical properties of membranes treated with C5b-8 and C5b-9, though in both cases the enhanced bilayer permeability is best attributed to the formation of trans-membrane channels. Collectively, the data are consistent with the hypothesis that the mechanism of membrane action by complement involves the production of a stable channel across the lipid bilayer, resulting in cell death by colloid-osmotic lysis. (+info)Molecular aspects of complement-mediated bacterial killing. Periplasmic conversion of C9 from a protoxin to a toxin. (5/225)
As part of the membrane attack complex complement protein C9 is responsible for direct killing of bacteria. Here we show that in the periplasmic space of an Escherichia coli cell C9 is converted from a protoxin to a toxin by periplasmic conditions missing in spheroplasts. This conversion is independent of the pathway by which C9 enters the periplasm. Both, C9 shocked into the periplasm and plasmid-expressed C9 targeted to the periplasm via a signal sequence are toxic. Toxicity requires disulfide-linked C9 because export into the periplasm of cells defective in disulfide bond synthesis (dsbA and dsbB mutants) is not toxic unless N-acetylcysteine is added externally to promote cystines. A N-terminal fragment, C9[1-144], is not toxic nor is cytoplasmically expressed C9, even in trxB mutants that are able to form disulfide bonds in the cytoplasm. Importantly, expression of full-length C9 in complement-resistant cells has no effect on their viability. Expression and translocation into the periplasm may provide a novel model to identify molecular mechanisms of other bactericidal disulfide-linked proteins and to investigate the nature of bacterial complement resistance. (+info)Acute phase proteins and C9 in patients with Behcet's syndrome and aphthous ulcers. (6/225)
Estimation of the concentration of C9, C-reactive protein (CRP) and alpha1-antitrypsin in forty sera from patients with Behcet's syndrome and recurrent oral ulcers showed significantly increased amounts of C9 and CRP in Behcet's syndrome. The concentration of C9 was also significantly raised in recurrent oral ulceration, though to a lesser extent than in Behcet's syndrome. The assay C9 and CRP might be useful in the differential diagnosis of Behcet's syndrome, especially from recurrent oral ulcers. It is suggested that during epithelial inflammation in recurrent oral ulcers some of the acute phase proteins are increased and in some patients these may modulate the immunological mechanism in such a way as to induce a transition from focal oral ulceration to the multifocal Behcet's syndrome. (+info)Free radicals upregulate complement expression in rabbit isolated heart. (7/225)
Both free radicals and complement activation can injure tissue. Our study determined whether free radicals alter complement production by the myocardium. Isolated hearts from New Zealand White rabbits were perfused on a Langendorff apparatus and exposed to xanthine (X; 100 microM) plus xanthine oxidase (XO; 8 mU/ml) (X/XO). The free radical-generating system significantly (P < 0.05) increased C1q and also increased C1r, C3, C8, and C9 transcription compared with controls. Immunohistological examination revealed augmented membrane attack complex deposition on X/XO-treated tissue. X/XO-treated hearts also exhibited significant (P < 0.05) increases in coronary perfusion pressure and left ventricular end-diastolic pressure and a decrease in left-ventricular developed pressure. N-(2-mercaptopropionyl)-glycine (3 mM), in conjunction with the superoxide dismutase mimetic SC-52608 (100 microM), significantly (P < 0.05) reduced the upregulation of C1q, C1r, C3, C8, and C9 mRNA expression elicited by X/XO. The antioxidants also ameliorated the deterioration in function caused by X/XO. Local complement activation may represent a mechanism by which free radicals mediate tissue injury. (+info)CD59 protects rat kidney from complement mediated injury in collaboration with crry. (8/225)
BACKGROUND: As previously reported, the membrane-bound complement regulator at the C3 level (Crry/p65) is important in maintaining normal integrity of the kidney in rats. However, the role of a complement regulator at the C8/9 level (CD59) is not clear, especially when activation of complement occurs at the C3 level. The aim of this work was to elucidate the in vivo role of CD59 under C3 activating conditions. METHODS: Two monoclonal antibodies, 5I2 and 6D1, were used to suppress the function of Crry and CD59, respectively. In order to activate alternative the pathway of complement, the left kidney was perfused with 5I2 and/or 6D1 and was recirculated. RESULTS: In the kidneys perfused with 5I2 alone, deposition of C3 and membrane attack complex (MAC) was observed in the peritubular capillaries, vasa recta, and tubular basement membranes. Cast formation, tubular dilation and degeneration, and cellular infiltration were observed at days 1 and 4, and they recovered by day 7. Further suppression of CD59 by 6D1 significantly enhanced the deposition of MAC and worsened the already exacerbated tubulointerstitial injury. These effects of 6D1 were dose dependent. Perfusion with 6D1 alone did not induce histologic damage or MAC deposition in the tubulointerstitium. CONCLUSIONS: In rats, CD59 maintains normal integrity of the kidney in collaboration with Crry in rats against complement-mediated damage in vivo. (+info)
Complement membrane attack complex - Wikipedia
CD59, an LY-6-like protein expressed in human lymphoid cells, regulates the action of the complement membrane attack complex on...
Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the...
The membrane attack complex of complement: Relation of C7 to the metastable membrane binding site of the intermediate complex...
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The membrane attack complex of complement: relation of C7 to the metastable membrane binding site of the intermediate complex...
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Quantitation of activation of the human terminal complement pathway by ELISA<...
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RCSB PDB
- 2QOS: Crystal structure of complement protein C8 in complex with a peptide containing the C8 binding site...
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Perforin
... has structural and functional similarities to complement component 9 (C9). Like C9, this protein creates transmembrane ... Shinkai Y, Takio K, Okumura K (1988). "Homology of perforin to the ninth component of complement (C9)". Nature. 334 (6182): 525 ... Granzymes Defensin Complement membrane attack complex GRCh38: Ensembl release 89: ENSG00000180644 - Ensembl, May 2017 GRCm38: ... Young JD, Cohn ZA, Podack ER (1986). "The ninth component of complement and the pore-forming protein (perforin 1) from ...
MACPF
"Nonsense mutation in exon 4 of human complement C9 gene is the major cause of Japanese complement C9 deficiency". Hum. Genet. ... Archetypal members of the family are complement C9 and perforin, both of which function in human immunity. C9 functions by ... Complement proteins C6-C9 all contain a MACPF domain and assemble into the membrane attack complex. C6, C7 and C8β appear to be ... 1997). "The human complement C9 gene: identification of two mutations causing deficiency and revision of the gene structure". J ...
Pore-forming toxin
Eukaryote MACPF proteins function in immune defence and are found in proteins such as perforin and complement C9. A family of ... Tschopp J, Masson D, Stanley KK (1986). "Structural/functional similarity between proteins involved in complement- and ...
Radang bahasa Indonesia, ensiklopedia bebas
A complex of the complement proteins C5b, C6, C7, C8, and multiple units of C9. The combination and activation of this range of ... Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. ... complement proteins forms themembrane attack complex, which is able to insert into bacterial cell walls and causes cell lysis ...
CD59
When complement activation leads to deposition of C5b678 on host cells, CD59 can prevent C9 from polymerizing and forming the ... Ninomiya H, Sims PJ (1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain ... Huang Y, Qiao F, Abagyan R, Hazard S, Tomlinson S (September 2006). "Defining the CD59-C9 binding interaction". J. Biol. Chem. ... 1992). "Complement regulatory proteins at the feto-maternal interface during human placental development: distribution of CD59 ...
Antibody-dependent enhancement
HIV-1 has also shown enhancement of infection in HT-29 cells when the viruses were pre-opsonized with complements C3 and C9 in ... Complement component receptors CR2, CR3 and CR4 have been found to mediate this Complement-mediated enhancement of infection. ... ADE in HIV can be complement-mediated or Fc receptor-mediated. Complements in the presence of HIV-1 positive sera have been ... The infection of HIV-1 leads to activation of complements. Fragments of these complements can assist viruses with infection by ...
Membranous glomerulonephritis
The immune complex serves as an activator that triggers a response from the C5b - C9 complements, which form a membrane attack ... Immunofluorescence microscopy will reveal typical granular deposition of immunoglobulins and complement along the basement ...
Outline of immunology
C6 C7 C8 C9 Complement pathway inhibitors C1-inhibitor - Classical, Lectin, Alternate Decay-accelerating factor (CD59) - ... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ... Classical complement pathway C1Q complex - C1R / C1S C4 - C4a C2 Mannan-binding lectin pathway MASP1 / MASP2 Mannan-binding ...
Meningococcal disease
Persons with component deficiencies in the final common complement pathway (C3, C5-C9) are more susceptible to N. meningitidis ... Orren A, Potter PC, Cooper RC, du Toit E (October 1987). "Deficiency of the sixth component of complement and susceptibility to ... In addition, complement component-deficient populations frequently experience frequent meningococcal disease since their immune ... Ross SC, Densen P; Densen (September 1984). "Complement deficiency states and infection: epidemiology, pathogenesis and ...
Membranous glomerulonephritis
C9 complements, which form a membrane attack complex (MAC) on the glomerular epithelial cells. This, in turn, stimulates ... Immunofluorescence microscopy will reveal typical granular deposition of immunoglobulins and complement along the basement ...
Meningococcal disease
C5-C9) are more susceptible to N. meningitidis infection than complement-satisfactory persons,[27][28][29][30][31][32][33] and ... Ross SC, Densen P; Densen (September 1984). "Complement deficiency states and infection: epidemiology, pathogenesis and ... Persons with component deficiencies in the final common complement pathway (C3, ... since their immune response to natural infection may be less complete than that of complement non-deficient persons.[27][35] ...
Alternative complement pathway
C5b binds sequentially to C6, C7, C8 and then to multiple molecules of C9 to form membrane attack complex. ... there are several different kinds of regulatory proteins that disrupt the complement activation process: *Complement Receptor 1 ... Complement Factor H can inhibit the formation of the C3 convertase by competing with factor B for binding to C3b;[1] accelerate ... "Inhibition of the alternative complement pathway by antisense oligonucleotides targeting complement factor B improves lupus ...
C9
... , C09 or C-9 may refer to: C9 (Complement component 9), a protein ATC code C09, a subgroup of the Anatomical Therapeutic ... C-9), a US Navy cruiser C9, the IATA code for Cirrus Airlines Sauber C9, a Le Mans racing car C9 engine, by Caterpillar Inc. C9 ... ICD-10 code for malignant neoplasm of tonsil Hi-Point Models C9 and C9 Comp handguns C9 LMG, Canadian light machine gun C9, an ... a note five octaves above Middle C C9, a C ninth chord Cloud9, an American esports organization C9 League, an association of ...
Complement membrane attack complex
Polymerization: C8-C9[edit]. Similar hydrophobic sites on C8 and C9 molecules are exposed when they bind to the complex, so ... C5b-6-7-8 subsequently binds to C9[4][5][6] and acts as a catalyst in the polymerization of C9. ... Multiple molecules of C9 can join spontaneously in concentrated solution to form polymers of C9. These polymers can also form a ... Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ...
Complement component 9
... (C9) is a MACPF protein involved in the complement system, which is part of the innate immune system. ... C9 is one member of the complement membrane attack complex (MAC), which also includes complement components C5b, C6, C7 and C8 ... C9 genes include 11 exons and 10 introns when found in fish. In fish, the liver is the site where the majority of complement ... Fu YW, Zhu CK, Zhang QZ (May 2019). "Molecular characterization and expression analysis of complement components C3 and C9 in ...
Complement control protein
CD59 for example blocks oligomerization of the complement peptide C9 stalling the formation of the Membrane Attack Complex (MAC ... Complement control protein are proteins that interact with components of the complement system. The complement system is ... Complement proteins protect against malignant cells- both by direct complement attack and through initiation of Complement- ... Complement C3b/C4b Receptor 1, CR1 (CD35) Complement Regulator of the Immunoglobulin Superfamily, CRIg Soluble complement ...
Total complement activity
The CH50 is testing the classical complement pathway in an individual thus requiring functioning C1-C9 factors. If an ... Total complement activity (TCA) refers to a series of tests that determine the functioning of the complement system in an ... Decreased CH50 values may be seen in cirrhosis or hepatitis as a result of impaired complement production in the liver. It can ... For example, if and individual has normal C3/C4 values but a decreased CH50, that can indicate a terminal complement pathway ...
Terminal complement pathway deficiency
Initial complement tests often include C3 and C4, but not C5 through C9. Instead, the CH50 result may play a role in diagnosis ... Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC). It ... While C9 is part of the MAC, and deficiencies have been identified, it is not required for cell lysis.) People with this ... Suspect terminal complement pathway deficiency with patients who have more than one episode of Neisseria infection. ...
Complement system
... and polymeric C9.[7] MAC is the cytolytic endproduct of the complement cascade; it forms a transmembrane channel, which causes ... complement factor B, and complement factor I, as well as deletion of complement factor H-related 3 and complement factor H- ... Complement deficiencyEdit. Main article: Complement deficiency. It is thought that the complement system might play a role in ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
Complement system
... and polymeric C9. MAC is the cytolytic endproduct of the complement cascade; it forms a transmembrane channel, which causes ... Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor ... The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...
CD9
Ninomiya H, Sims PJ (July 1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b" ... "domain of C9". The Journal of Biological Chemistry. 267 (19): 13675-80. PMID 1377690. Miyake M, Koyama M, Seno M, Ikeyama S ( ...
Complement component 1s
... (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... complement activation, classical pathway. • immune system process. • innate immune response. • ... Sim RB (1981). "The human complement system serine proteases C1r and C1s and their proenzymes". Methods in Enzymology. 80 Pt C ...
CCGS Tanu
Tanu has a complement of 15 composed of 6 officers and 9 crew, with berths for an additional 16. The vessel can be equipped ... The vessel is also equipped with three Caterpillar C9 generators and one Perkins 2430 emergency generator. ...
CD59 - 维基百科,自由的百科全书
The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain of C9. J. Biol. Chem. 1992, ... regulation of complement activation. • cell activation. • neutrophil degranulation. • regulation of complement-dependent ... complement binding. 細胞組分. • 囊泡. • endoplasmic reticulum membrane. • 膜. • 粘着斑. • Golgi membrane. • 细胞膜. • 細胞外區域. • cell surface ... Entrez Gene: CD59 molecule, complement regulatory protein.. *^ 7.0 7.1 Maio M, Brasoveanu LI, Coral S, Sigalotti L, Lamaj E, ...
Primary immunodeficiency
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1-inhibitor deficiency (hereditary angioedema) Factor I ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
Autoimmune hemolytic anemia
Cases may also arise with complement alone or with IgA, IgM or a combination of these three antibody classes and complement. ... C9) either can form the membrane attack complex (MAC) or can bind the antibody, aiding phagocytosis by macrophages (C3b). This ... Antibodies are produced against the RBCs, which leads to complement activation. Complement fragments, such as C3a, C4a and C5a ... IgM is a potent activator of the classical complement pathway, thus, AIHA involving IgM is characterized by complement mediated ...
Steyr M
In 2010 the C (Compact) sized C-A1 series was introduced in 9×19mm Parabellum and .40 S&W chamberings as the C9-A1 and C40-A1. ... In 2013 the Steyr M (Medium) and S (Small) form factors were complemented by the L (Large) sized series and the C (Compact) ... For Italy only the C9-A1 is offered in the 9×21mm chambering. In 2013 the Steyr M (Medium), Steyr C (Compact) and S (Sub- ... Third generation' Steyr C9-A1. 'Third generation' Steyr M9-A1. 'Third generation' Steyr L9-A1. 'Third generation' Steyr ...
List of primary immunodeficiencies
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C8a deficiency C8b deficiency C9 deficiency (Neisserial infections) C1-inhibitor deficiency (hereditary angioedema) Factor I ... Cherubism COPA defect Otulipenia/ORAS The complement system is part of the innate as well as the adaptive immune system; it is ... MASP2 deficiency Complement receptor 3 deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex inhibitor ...
Komplemendisüsteem - Vikipeedia
Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... Üheks selliseks näiteks on CD59 valk (protektiin), mis inhibeerib membraaniründe kompleksi moodustumise ajal C9 valgu ... 1,0 1,1 1,2 1,3 Rus, H., Cudrici, C., & Niculescu, F. (2005). The role of the complement system in innate immunity. Immunologic ... 7,0 7,1 Lambris, J. D., Ricklin, D., & Geisbrecht, B. V. (2008). Complement evasion by human pathogens. Nature Reviews. ...
FOXA3
Corrocher R, Tedesco F, Rabusin P, De Sandre G (1975). "Effect of human erythrocyte stromata on complement activation". Br. J. ...
Behavioral economics
It complements "rationality as optimization", which views decision-making as a fully rational process of finding an optimal ... Relevant subcategories are found at the Journal of Economic Literature classification codes at JEL: C9. ...
Jewel (supermarket)
p. C9, C12.. Alternate Link via ProQuest.. *^ "Jewel's Grand Bazaar draws 30,000 weekly". Chicago Tribune. March 20, 1974. p. ... The acquisition of both Osco and Turn Style allowed Jewel to expand into non-food related retailing that would complement their ... to complement their food store division when building one-stop shopping destinations, such as the new Family Centers and Jewel- ...
Porenbildendes Toxin
Eukaryotische MACPF-ähnliche Proteine kommen beim Perforin und dem C9 des Komplementsystems als Teil der Immunabwehr vor.[3] ... Structural/functional similarity between proteins involved in complement- and cytotoxic T-lymphocyte-mediated cytolysis. . In: ...
C5a receptor
Sengeløv H (1996). "Complement receptors in neutrophils.". Crit. Rev. Immunol. 15 (2): 107-31. PMID 8573284. ... MAC (C6, C7, C8, C9). Inhibitori. CLA: C1-inhibitor - Faktor ubrzanja raspada/CD59 - Faktor I CL: C4BP ... "Function, structure and therapeutic potential of complement C5a receptors". British Journal of Pharmacology 152 (4): 429-48. ...
Sîstema temamker - Wîkîpediya
C5 bi proteînên C6, C7, C8 û C9 an ve li ser rûyê hokara nexweşiyê de xelekek ava dikin. Di nav xelekê de C9 hê pirtir kom dibe ... a b https://www.britannica.com/science/complement-immune-system-component *↑ a b c d e f g h i OpenStax, Microbiology, openstax ... Sîstema temamker an jî sîstema tewawker (bi îngilîzî: complement system)[1] beşek ji sîstema bergiriya giştî ye. Sîstema ... Xeleka ji proteînên C6, C7, C8, û C9 ê pêk tê wekî pêkhateya êrîşa parzûnê bi nav dibe (bi îngilîzî: membrane attack complex ( ...
McDonnell Douglas DC-9
During the 1950s Douglas Aircraft studied a short- to medium-range airliner to complement their higher capacity, long range DC- ... McDonnell Douglas C-9. Developed into. McDonnell Douglas MD-80. McDonnell Douglas MD-90. Boeing 717. ...
Basigin
... is a member of the immunoglobulin superfamily, with a structure related to the putative primordial form of the family. As members of the immunoglobulin superfamily play fundamental roles in intercellular recognition involved in various immunologic phenomena, differentiation, and development, basigin is thought also to play a role in intercellular recognition (Miyauchi et al., 1991; Kanekura et al., 1991).[9][10] It has a variety of functions. In addition to its metalloproteinase-inducing ability, basigin also regulates several distinct functions, such as spermatogenesis, expression of the monocarboxylate transporter and the responsiveness of lymphocytes.[6] Basigin is a type I integral membrane receptor that has many ligands, including the cyclophilin (CyP) proteins Cyp-A and CyP-B and certain integrins.[11][12][13] It is expressed by many cell types, including epithelial cells, endothelial cells and leukocytes. The human basigin protein contains 269 amino acids that form two heavily ...
Pedal d'efectes - Viquipèdia, l'enciclopèdia lliure
Un pedal d'efectes (o, per simplificació, pedal de distorsió) és un complement per a tocar la guitarra elèctrica i altres ... C9. sim.. 9 sons d'orgue EHX. Canyon. repe.. delay i looper 2018. Ammoon. PockEcho. repe.. delay/looper ...
Perforina, a enciclopedia libre
A perforina ten semellanzas estruturais e funcionais co compoñente do complemento 9 (C9). Igual que C9, esta proteína crea ... Tschopp J, Masson D, Stanley KK (1986). "Structural/functional similarity between proteins involved in complement- and ...
Sistem komplementa - Wikipedija, prosta enciklopedija
Označujemo jih z veliko črko C (kratica za complement) in številko. Ko signal sproži spremembo enega od začetnih proencimov v ... Tam sproži polimerizacijo 10 do 16 molekul C9 v obročasto strukturo, imenovano »kompleks, ki napada membrano« (membrane attack ...
Receptor (biochemistry)
... complement receptors, Fc receptors, B cell receptors and T cell receptors.[12] ...
Pattern recognition receptor
C5, C6, C7, C8 and C9 form the membrane attack complex (MAC). This is another large superfamily of CLRs that includes The ... Complement receptors, collectins, ficolins, pentraxins such as serum amyloid and C-reactive protein, lipid transferases, ... Once bound to the ligands MBL and Ficolin oligomers recruit MASP1 and MASP2 and initiate the lectin pathway of complement ... "/"self turned nonself" type pathogen pattern are also identified and destroyed (e.g. by complement fixation or other cytotoxic ...
HealthLine
Complementing the HealthLine is a set of bike lanes on the outer edges of the stretch Euclid Avenue that connects Cleveland ... A low sulfur diesel engine (Caterpillar C-9) generates electrical power to run smaller electric motors mounted on each of the ...
Beijing
... is home to the two best C9 League universities (Tsinghua and Peking) in the Asia-Pacific and emerging countries. ... one of four secondary capitals to complement its "Supreme Capital", Shangjing (modern Baarin Left Banner in Inner Mongolia). ... Both are members of the C9 League, an alliance of elite Chinese universities offering comprehensive and leading education. The ... "Eastern stars: Universities of China's C9 League excel in select fields". Times Higher Education (THE). 17 February 2011. ...
Unlocked (album)
"The two artists complement each other effortlessly making Unlocked a collaboration that hip hop didn't know it needed in 2020 ... Madden, Sidney (November 15, 2016). "Meet Every Member of Denzel Curry's C9 Collective". XXL. Archived from the original on ...
Lex Papia Poppaea
c9; compare Satire IX of Juvenal, line 84). The law also imposed penalties on orbi, that is, married persons who had no ... It included provisions against adultery and against celibacy after a certain age and complemented and supplemented Augustus' ...
List of MeSH codes (D12.776.124)
... complement c7 MeSH D12.776.124.486.274.750 - complement c8 MeSH D12.776.124.486.274.850 - complement c9 MeSH D12.776.124.486. ... complement c1 MeSH D12.776.124.486.274.050.270 - complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776. ... complement c2 MeSH D12.776.124.486.274.150.500 - complement c2a MeSH D12.776.124.486.274.150.750 - complement c2b MeSH D12.776. ... complement c3c MeSH D12.776.124.486.274.250.260.750 - complement c3d MeSH D12.776.124.486.274.350 - complement c4 MeSH D12.776. ...
McDonnell Douglas DC-9
During the 1950s Douglas Aircraft studied a short- to medium-range airliner to complement their higher capacity, long range DC- ... ISBN 0-354-00538-3. Boeing: Historical Snapshot: DC-9/C-9 Transport "DC-9 history page on Boeing.com". Archived from the ... 25,596 kg Aviation portal 2006 Mexico DC-9 drug bust Airstair Related development Boeing 717 Comac ARJ21 McDonnell Douglas C-9 ...
Complement component C9 (P79755) | InterPro | EMBL-EBI
Complement C9 Antibody
Complement C9 Polyclonal Antibody from Invitrogen for Western Blot and Immunohistochemistry (Paraffin) applications. This ... Cite Complement C9 Polyclonal Antibody. The following antibody was used in this experiment: Complement C9 Polyclonal Antibody ... Protein Aliases: C5b-9 complex component; Complement component C9; Membrane Attack Complex component ... PA5-19826 targets C9 in IHC (P) and WB applications and shows reactivity with Human samples.. The PA5-19826 immunogen is ...
Complement Component C9 Lysates: Novus Biologicals
Browse our Complement Component C9 Lysate catalog backed by our Guarantee+. ... Complement Component C9 Lysates available through Novus Biologicals. ... Complement Component C9 Lysates. We offer Complement Component C9 Lysates for use in common research applications: Western Blot ... Our Complement Component C9 Lysates can be used in a variety of model species. Use the list below to choose the Complement ...
C9 - Complement component C9 precursor - Homo sapiens (Human) - C9 gene & protein
C9 is the pore-forming subunit of the MAC (PubMed:4055801, PubMed:26841934, PubMed:30111885). ... "The architecture of complement component C9 and poly(C9).". DiScipio R.G., Hugli T.E.. J. Biol. Chem. 260:14802-14809(1985) [ ... "The architecture of complement component C9 and poly(C9).". DiScipio R.G., Hugli T.E.. J. Biol. Chem. 260:14802-14809(1985) [ ... "The architecture of complement component C9 and poly(C9).". DiScipio R.G., Hugli T.E.. J. Biol. Chem. 260:14802-14809(1985) [ ...
Complement C9 antibody | acris-antibodies.com
The three distinct activation pathways of complement converge with the formation of a C5 convertase. The cleavage of C5 by this ... Background of Complement C9 antibody. The three distinct activation pathways of complement converge with the formation of a C5 ... Immunohistochemical analysis of Complement C9 staining in human liver cancer formalin fixed paraffin embedded tissue section. ... Lane 1 : Complement factor C9 isolated from human plasma. Lane 2 : Human plasma.. *MAB0780 ...
Complement Component C9 - 产品: Leica Biosystems
Complement component C9 acts in a similar way to perforin, a pore forming protein found in cytotoxic T cells. Male and female ... Complement component C9 binds to the C5b-8 complex as the final protein of the membrane attack complex. After binding, it ... The detection of complement component C9 has been reported in cases of acute myocardial damage at necropsy. Detection of ... Human myocardium: immunohistochemical staining for complement component C9 using NCL-CCC9. Note staining of partially necrotic ...
Complement C9 Antibody (FITC) for IHC, WB/Western LS-C693053
Complement C9 antibody LS-C693053 is an FITC-conjugated guinea pig polyclonal antibody to rabbit Complement C9. Validated for ... C9 Antibody, Complement C9 Antibody, Complement component 9 Antibody, Complement component C9 Antibody ... Complement C9 antibody LS-C693053 is an FITC-conjugated guinea pig polyclonal antibody to rabbit Complement C9. Validated for ... Complement C9 antibody LS-C693053 is an FITC-conjugated guinea pig polyclonal antibody to rabbit Complement C9. Validated for ...
Complement C9, Human CAS 80295-59-6 | 204910
Complement C9, Human, CAS 80295-59-6, is a native, single-chain glycoprotein present in normal human serum at 60 µg/ml. - Find ... human C9 complement component. Single-chain glycoprotein present in normal human serum at 60 µg/ml. On activation of complement ... human C9 complement component. Single-chain glycoprotein present in normal human serum at 60 µg/ml. On activation of complement ... Complement C9, Human, CAS 80295-59-6, is a native, single-chain glycoprotein present in normal human serum at 60 µg/ml.. More,, ...
Complement C9 Antibody (aa22-265, HRP) for IHC, WB/Western LS-C710425
Complement C9 antibody LS-C710425 is an HRP-conjugated rabbit polyclonal antibody to human Complement C9. Validated for IHC and ... C9 Antibody, Complement C9 Antibody, Complement component 9 Antibody, Complement component C9 Antibody ... Complement C9 antibody LS-C710425 is an HRP-conjugated rabbit polyclonal antibody to human Complement C9. Validated for IHC and ... Complement C9 antibody LS-C710425 is an HRP-conjugated rabbit polyclonal antibody to human Complement C9. Validated for IHC and ...
Monoclonal antibody targeting complement C9 binding domain of Trichinella spiralis paramyosin impairs the viability of...
Previous studies demonstrated that Ts-Pmy bound to complement components C8 and C9 and inhibited the polymerization of C9 ... The binding of mAb 9G3 to Ts-Pmy efficiently blocked the binding of Ts-Pmy to human complement C9, resulting in a significant ... mAb 9G3 is a specific antibody that binds to the C9 binding domain of Ts-Pmy and interferes with Ts-Pmys complement-binding ... In this study, a monoclonal antibody against the complement C9 binding domain of Ts-Pmy (mAb 9G3) was produced using hybridoma ...
Complement Component C9 - 製品情報: Leica Biosystems
Complement component C9 binds to the C5b-8 complex as the final protein of the membrane attack complex. After binding, it ... Complement Component C9 Primary Antibodies Complement Component C9 Novocastra. *製品情報 ... Complement component C9 acts in a similar way to perforin, a pore forming protein found in cytotoxic T cells. Male and female ... Complement component C9 binds to the C5b-8 complex as the final protein of the membrane attack complex. After binding, it ...
reagents-immunoassay-protein-complement-component-c5-c9
Deficiency of complement components C5 to C9 are associated with several diseases, especially recurrent neisserial infections. ... IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C5-C9. Definition : Immunoassay reagents intended to perform ... Home > Specialties > IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C5-C9 ... Entry Terms : "C5-9 (Complement Component) Determination Reagents" , "Reagents, Immunoassay, Protein, Complement Component, C5- ...
Complement C9 deficiency
... Complement component 9 deficiency is caused by mutations of the C9 gene. If both alleles are altered ... Recurrent meningitis in a patient with congenital deficiency of the C9 component of complement. First case of C9 deficiency in ... Abbott C et al. (1989) The gene for human complement component C9 mapped to chromosome 5 by polymerase chain reaction. ... Witzel-Schlömp K et al. (1997) The human complement C9 gene: identification of two mutations causing deficiency and revision of ...
Association between a non-synonymous single nucleotide polymorphism in the Complement component 9 (C9) gene and meat-quality...
C9) gene was identified, and the association between meat quality traits and the C9 genotype was analyzed. The nsSNP in the C9 ... 그 결과, C9 유전자의 cDNA 942번 G 서열이 T 서열로 변환되어 라이신(lysin)이 아스파라진(asparagin)으로 변하는 non-synonymous SNP를 확인하였다. Berkshire 돼지 405두에서 C9 ... 가 증가되고 육즙 손실이 감소되는 것으로 확인되어 C9 유전자의 TG 유전자를 가진 돼지가 더 좋은 육질을 가지는 것으로 판단된다. 본 결과를 바탕으로 C9 유전자의 단일염기다형성을 육질을 판단하는 생물마커(biomarker) ... 성별에 따른 C9 유전자형과 육질 형질 간의 연관성을 분석한 결과 거세돈에서 도체중, 콜라겐에서 유의성이 있었으며, 암퇘지의 경우 육색의 명도, 단백질, ,TEX,$pH_{24h}$,/TEX, 육질 형질에서 유의성이 있었다. ...
C9 Complement, Functional, Serum -
Munson Healthcare Laboratories
Test Code C9FX C9 Complement, Functional, Serum Reporting Name. C9 Complement, Functional, S ... The total complement (CH50) assay (COM / Complement, Total, Serum) should be used as a screen for suspected complement ... A deficiency of an individual component of the complement cascade will result in an undetectable total complement level. ... Investigation of a patient with a low total (hemolytic) complement (CH50) level ...
Complement C9 Blood Test - Private Blood Tests London
anti-Complement Factor C9 mAb (64E9) - Order from Adipochem
anti-Complement Factor C9, mAb (64E9) is a monoclonal antibody that crossreacts with human protein. Works in WB. Important for ... Complement factor C9, 71kDa, is the last of the human complement components creating the membrane attack complex (MAC). ... Such lytic action could be the basis for complement autotoxicity. Like most of the complement components, C9 belongs to the ... Also, because C9 is required for the MAC of complement to become functional, interfering with signaling pathways that stimulate ...
R-PLEX Human Complement C9 Antibody Set | Meso Scale Discovery
R-PLEX Human Complement C9 Antibody Set Calibration Curve. Complement C9 Analyte. Description - Complement C9 (C9D, ARMD15, C9 ... R-PLEX Human Complement C9 Antibody Set .testRadioBox { padding-left:40%; } R-PLEX Human Complement C9 Antibody Set Overview. ... R-PLEX Human Complement C9 Antibody Set Features. *Measures human Complement C9 in plasma ... Calibration curve for the R-PLEX Human Complement C9 Antibody Set was assessed and representative data is presented below. ...
Deposition of complement C9 in primary sclerosing cholangitis (PSC). - Experimental Medicine Division
iTRAQ-Based Quantitative Proteomic Analysis Identified HSC71 as a Novel Serum Biomarker for Renal Cell Carcinoma
Patent US6891154 - Amino acid sequence pattern matching - Google Patents
Patente US3625214 - Drug-delivery device - Google Patentes
Terminal component of complement (C9) in the cerebrospinal fluid of patients with multiple sclerosis and neurologic controls<...
Terminal component of complement (C9) in the cerebrospinal fluid of patients with multiple sclerosis and neurologic controls. ... Terminal component of complement (C9) in the cerebrospinal fluid of patients with multiple sclerosis and neurologic controls. ... Terminal component of complement (C9) in the cerebrospinal fluid of patients with multiple sclerosis and neurologic controls. ... Fingerprint Dive into the research topics of Terminal component of complement (C9) in the cerebrospinal fluid of patients with ...
Proteolysis of the monomeric and dimeric C5b-9 complexes of complement: Alteration in the susceptibility to proteases of the C9...
The uncomplexed C9 was also cleaved by both enzymes at multiple sites. By contrast, the C9 subunits of the C5b-9 dimer were ... The uncomplexed C9 was also cleaved by both enzymes at multiple sites. By contrast, the C9 subunits of the C5b-9 dimer were ... The uncomplexed C9 was also cleaved by both enzymes at multiple sites. By contrast, the C9 subunits of the C5b-9 dimer were ... The uncomplexed C9 was also cleaved by both enzymes at multiple sites. By contrast, the C9 subunits of the C5b-9 dimer were ...
Rat C9(Complement Component 9) ELISA Kit - Rat C9(Complement Component 9) ELISA Kit Exporter, Importer, Manufacturer,...
Complement Component 9) ELISA Kit based in Delhi, India ... Rat C9(Complement Component 9) ELISA Kit. *Rat C4a(Complement ... The OD value is proportional to the concentration of Rat C9. You can calculate the concentration of Rat C9 in the samples by ... This kit recognizes Rat C9 in samples. No significant cross-reactivity or interference between Rat C9 and analogues was ... The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to Rat C9. Standards or samples are ...
Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the...
Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the ... Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the ... Molecular organization of C9 within the membrane attack complex of complement. Induction of circular C9 polymerization by the ... When C9 input was restricted so that the molar C9/C8 ratio was ≤3, C9-induced aggregates of C5b-8 were observed but virtually ...
Proteoform Profile Mapping of the Human Serum Complement Component C9
Revealing Unexpected New Features of N‑, O‑, and C...
Proteoform Profile Mapping of the Human Serum Complement Component C9 Revealing Unexpected New Features of N‑, O‑, and C‑ ... The human complement C9 protein (∼65 kDa) is a member of the complement pathway. It plays an essential role in the membrane ... Human Serum Complement Component C 9 RevealingSubsequent peptide-centric analysisN-X-SMACporemass spectrometrycomplementMSC 9. ... Human Serum Complement Component C 9 RevealingSubsequent peptide-centric analysisN-X-SMACporemass spectrometrycomplementMSC 9. ...
Low-density lipoprotein (LDL) receptor class A, conserved site (IPR023415) | InterPro | EMBL-EBI
MRMaid, the Web-based Tool for Designing Multiple Reaction Monitoring (MRM) Transitions | Molecular & Cellular Proteomics
How to Dig Deeper? Improved Enrichment Methods for Mucin Core-1 Type Glycopeptides | Molecular & Cellular Proteomics
ProteinsDeficiencyProteinMembraneSerumDeficienciesAntibodyPathwayLate-acting complement comCascadeMoleculesInflammationDepositionImmunologyPerforinLectinParoxysmal nocturnal hemoglobinuria withSusceptibilityLysisActivationComponentsReactivityFunctionalComponent of the complementHuman C9AntibodiesInnatePathogensSystemConcentrationGenesLevels of complementSpeciesMolecularPorcineTotal complementMACPFDiminished third component of complementInhibitorImmunohistochemicalImmunePore
Proteins37
- Male and female reproductive tissues express and synthesize complement components, binding proteins and receptors, although the implications of this is unclear. (leicabiosystems.com)
- Subsequent studies revealed that T. spiralis worms could bind to complement components [ 8 - 10 ], suggesting that T. spiralis contains proteins that bind to and potentially inhibit complement activation to protect against host complement attack. (biomedcentral.com)
- Immunoassay reagents intended to perform qualitative and/or quantitative analyses on a body fluid sample (typically serum) to detect and/or measure levels of one or more of the proteins C5 to C9 found in the final complement pathway. (ophthalmologymanagement.com)
- Like most of the complement components, C9 belongs to the acute-phase proteins which are generally of hepatic origin, and their plasma concentrations increase (or decrease) following tissue injury and inflammation. (adipogen.com)
- In mixture, the two proteins formed a reversible equimolar complex that had a sedimentation coefficient of 10.5S. In contrast, a single, cell-bound C5b-8 complex was found to bind up to 12-15 C9 molecules and clusters of C5b-8 bound 6-8 C9 molecules per C8 molecule. (elsevier.com)
- Genes that encode the proteins of complement components or their isotypes are distributed throughout different chromosomes, with 19 genes comprising 3 significant complement gene clusters in the human genome. (medscape.com)
- The important components of this system are various cell membrane-associated proteins such as complement receptor 1 (CR1), complement receptor 2 (CR2), and decay accelerating factor (DAF). (medscape.com)
- The membrane attack complex ( MAC ) or terminal complement complex ( TCC ) is a structure typically formed on the surface of pathogen cell membranes as a result of the activation of the host's complement system , and as such is one of the effector proteins of the immune system . (wikipedia.org)
- MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma membrane , and many copies of a fifth protein (C9) that hook up to one another, forming a ring in the membrane. (wikipedia.org)
- The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells. (medlineplus.gov)
- The complement proteins work with your immune system to protect the body from infections. (medlineplus.gov)
- There are nine major complement proteins. (medlineplus.gov)
- Rarely, people may inherit deficiency of some complement proteins. (medlineplus.gov)
- Hereditary deficiency in one of the complement proteins will usually lead to a high frequency of recurrent microbial infections. (labcorp.com)
- Complement protein levels are usually increased, along with other unrelated proteins called acute phase reactants, during acute or chronic inflammation. (labcorp.com)
- Of these proteins, we selected periplakin, envoplakin, villin-1, and complement C3 and C9 for confirmation because they were also significantly overrepresented in pathway analysis and were previously implicated in ADPKD pathogenesis. (asnjournals.org)
- In summary, proteomic analysis of uEVs identified plakins and complement as disease-associated proteins in ADPKD. (asnjournals.org)
- Complement is a blood test that measures the activity of certain proteins in the liquid portion of your blood. (medlineplus.gov)
- For example, people with active lupus erythematosus may have lower-than-normal levels of the complement proteins C3 and C4 . (medlineplus.gov)
- The cascade activates the complement proteins. (medlineplus.gov)
- The combination and activation of this range of complement proteins forms the membrane attack complex , which is able to insert into bacterial cell walls and causes cell lysis with ensuing death. (wikipedia.org)
- Vertebrate complement proteins C6 to C9. (embl.de)
- When the complement system is turned on during inflammation, levels of complement proteins may go down. (ucsfhealth.org)
- The complement system consists of a number of small proteins found in the blood, in general synthesized by the liver , and normally circulating as inactive precursors ( pro-proteins ). (wikipedia.org)
- Over 30 proteins and protein fragments make up the complement system, including serum proteins , and cell membrane receptors . (wikipedia.org)
- Most of the proteins and glycoproteins that constitute the complement system are synthesized by hepatocytes . (wikipedia.org)
- C6-C9 or serum proteins is not present. (mpbio.com)
- The complement system is a group of proteins that defend against infection and play a role in the development of inflammation. (lifeextension.com)
- There are nine major complement proteins called C1 through C9. (lifeextension.com)
- Complement resistance in Borrelia burgdorferi strain 297: outer membrane proteins prevent MAC formation at lysis susceptible sites. (nih.gov)
- The Classical pathway of activation of the complement system is a group of blood proteins that mediate the specific antibody response. (wikipathways.org)
- This unit describes several assay methods that can be used to determine the functional status of the classical pathway of complement and to quantitate its component proteins. (currentprotocols.com)
- These can be used to measure the concentrations of most circulating complement proteins and to evaluate the functional status of C1‐esterase inhibitor. (currentprotocols.com)
- The platelet also exhibits physiological responses to activated proteins of another cascade pathway, complement. (springer.com)
- Ando B, Wiedmer T, Hamilton KK, Sims PJ (1988) Complement proteins C5b-9 initiate secretion of platelet storage granules without increased binding of fibrinogen or von Willebrand factor to newly expressed cell surface GPIIb-llla. (springer.com)
- Ando B, Wiedmer T, Sims PJ (1989) The secretory release reaction initiated by complement proteins C5b-9 occurs without platelet aggregation through GPIIb-llla. (springer.com)
- Houle JJ, Leddy JP, Rosenfield SI (1989) Secretion of the terminal complement proteins, C5-C9, by human platelets. (springer.com)
Deficiency29
- Mutations in this gene cause component C9 deficiency. (thermofisher.com)
- Deficiency of complement components C5 to C9 are associated with several diseases, especially recurrent neisserial infections. (ophthalmologymanagement.com)
- Complement component 9 deficiency is caused by mutations of the C9 gene. (moldiag.com)
- The clinical manifestations seem to be tempered in paroxysmal nocturnal hemoglobinuria with complement component 9 deficiency. (moldiag.com)
- C9 deficiency has a frequency of about 2/1000 in Japan. (moldiag.com)
- Recurrent meningitis in a patient with congenital deficiency of the C9 component of complement. (moldiag.com)
- First case of C9 deficiency in Europe. (moldiag.com)
- A deficiency of an individual component of the complement cascade will result in an undetectable total complement level. (testcatalog.org)
- Defects in C9 are a cause of complement component 9 deficiency (C9D) [MIM:613825]. (abcam.com)
- Complement component 9 deficiency (C9D) [MIM:613825]: A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (avivasysbio.com)
- Cephalosporins are often used for treatment of N meningitidis infection in patients with complement deficiency. (medscape.com)
- Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. (medscape.com)
- Molecular basis of complement factor I deficiency in Tunisian atypical hemolytic and uremic syndrome Patients. (medscape.com)
- Arnold DF, Roberts AG, Thomas A, Ferry B, Morgan BP, Chapel H. A novel mutation in a patient with a deficiency of the eighth component of complement associated with recurrent meningococcal meningitis. (medscape.com)
- C5 Complement Deficiency in a Saudi Family, Molecular Characterization of Mutation and Literature Review. (medscape.com)
- Invasive meningococcal disease in three siblings with hereditary deficiency of the 8(th) component of complement: evidence for the importance of an early diagnosis. (medscape.com)
- Complement C4 deficiency--a plausible risk factor for non-tuberculous mycobacteria (NTM) infection in apparently immunocompetent patients. (medscape.com)
- The Brief Case: Meningococcemia Leading to a Diagnosis of Complement Deficiency in a 23-Month-Old. (medscape.com)
- Cases of complement deficiency have helped defined the role of complement in host defense. (medscape.com)
- A North African study of molecular basis of complement factor I deficiency in atypical hemolytic and uremic syndrome patients suggested that the Ile357Met mutation may be a founding effect. (medscape.com)
- The clinical impact of a deficiency in C9 is an infection with the gram-negative bacterium Neisseria meningitidis. (wikipedia.org)
- Mutations in this gene are associated with complement component-6 deficiency. (genecards.org)
- Diseases associated with C6 include Complement Component 6 Deficiency and Immunodeficiency Due To A Late Component Of Complement Deficiency . (genecards.org)
- Defects in C7 are a cause of complement component 7 deficiency (C7D) [MIM:610102]. (abcam.com)
- Complement levels may be decreased due to increased consumption or, more rarely, a hereditary deficiency. (labcorp.com)
- If the deficiency is due to an underlying acute or chronic condition, complement levels will usually return to normal if the underlying condition can be resolved. (labcorp.com)
- Human ortholog(s) of this gene implicated in age related macular degeneration 15 and complement component 9 deficiency. (zfin.org)
- Human deficiency of the fifth complement component (C5D) or complete absence of the sixth component (C6Q0) leads to increased risk of MD, which is often recurrent. (scielo.org.za)
- We strongly recommend diagnostic testing for complement C5 and C6 deficiency in the routine work-up of all MD cases in South Africa. (scielo.org.za)
Protein21
- Complement component C9 binds to the C5b-8 complex as the final protein of the membrane attack complex. (leicabiosystems.com)
- Complement component C9 acts in a similar way to perforin, a pore forming protein found in cytotoxic T cells. (leicabiosystems.com)
- Trichinella spiralis expresses paramyosin ( Ts -Pmy) not only as a structural protein but also as an immunomodulator that inhibits host complement as a survival strategy. (biomedcentral.com)
- The nsSNP in the C9 gene was located at c.942 G>T. In the dominant model, significant associations were observed between the SNP and meat quality traits such as CIE L, collagen content, moisture level, and $pH_{24h}$ , whereas in the co-dominant model, significant associations were observed between the SNP and CIE L, collagen content, and protein content. (koreascience.or.kr)
- The human complement C9 protein (∼65 kDa) is a member of the complement pathway. (figshare.com)
- The membrane attack complex is initiated when the complement protein C5 convertase cleaves C5 into C5a and C5b. (wikipedia.org)
- Another complement protein, C6 , binds to C5b. (wikipedia.org)
- Like C9, this protein creates transmembrane tubules and is capable of lysing non-specifically a variety of target cells. (wikipedia.org)
- Complement component 9 (C9) is a MACPF protein involved in the complement system, which is part of the innate immune system. (wikipedia.org)
- C9 was found to be the most strongly under expressed serum protein in men who achieved longevity, compared to men who did not. (wikipedia.org)
- MACPF protein, terminal complement component, TCC-like of 585 aas. (tcdb.org)
- C6 (Complement C6) is a Protein Coding gene. (genecards.org)
- It controls a protein called C1, which is part of the complement system. (medlineplus.gov)
- Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. (wikipedia.org)
- Complement component 4 is a blood test that measures the activity of a certain protein. (ucsfhealth.org)
- This protein is part of the complement system. (ucsfhealth.org)
- Her binder C1q antistoffer eller andre molekyler, for eksempel C-reaktivt protein, som er bundet på en overflate. (tidsskriftet.no)
- Complement component C3 is the most important and abundant protein in the complement system. (lifeextension.com)
- Download the full report: https://www.reportbuyer.com/product/4368352 Summary Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) - Complement component 5 is a protein is encoded by the C5 gene. (medindia.net)
- C5a is also a potent chemokine which stimulates the locomotion of polymorphonuclear leukocytes and directs their migration toward sites of inflammation.Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) pipeline Target constitutes close to 31 molecules. (medindia.net)
- It also reviews key players involved in Complement C5 (C3 And PZP Like Alpha 2 Macroglobulin Domain Containing Protein 4 or C5) targeted therapeutics development with respective active and dormant or discontinued projects.The report is built using data and information sourced from proprietary databases, company/university websites, clinical trial registries, conferences, SEC filings, investor presentations and featured press releases from company/university sites and industry-specific third party sources. (medindia.net)
Membrane25
- C9 neoantigens are present both in the membrane-bound (MAC) and the fluid-phase (SC5b-9) complex. (acris-antibodies.com)
- On activation of complement via either the classical or alternative pathway, formation of the C5b fragment initiates assembly of the C5b-9 complement membrane attack complex (MAC) on target surfaces. (emdmillipore.com)
- Previous studies demonstrated that Ts -Pmy bound to complement components C8 and C9 and inhibited the polymerization of C9 during the formation of the membrane attack complex (MAC). (biomedcentral.com)
- Complement factor C9, 71kDa, is the last of the human complement components creating the membrane attack complex (MAC). (adipogen.com)
- Molecular organization of C9 within the membrane attack complex of complement. (elsevier.com)
- Evidence has been presented suggesting that during assembly of the membrane attack complex (MAC) of complement, the C5b-8 complex induces polymerization of C9. (elsevier.com)
- In either case, typical ultrastructural membrane lesions were observed, suggesting that the membrane lesion is identical with the tubular poly C9 consisting of 12-16 C9 molecules, and that the MAC can have either the composition (C5b-8)polyC9 or (C5b-8) 2 polyC9. (elsevier.com)
- When C9 input was restricted so that the molar C9/C8 ratio was ≤3, C9-induced aggregates of C5b-8 were observed but virtually no circular membrane lesions were found. (elsevier.com)
- We suggest, therefore, that C9, at low dosage, causes cross-linking of multiple C5b-8 complexes within the target membrane and that, at high dosage, C9 is polymerized by C5b-8 to form a transmembrane channel within the MAC assembly. (elsevier.com)
- It is primarily the C9 polymer that evokes the ultrastructural image of the MAC or of membrane lesions caused by complement. (elsevier.com)
- Fingerprint Dive into the research topics of 'Molecular organization of C9 within the membrane attack complex of complement. (elsevier.com)
- The ring structure formed by C9 is a pore in the membrane that allows free diffusion of molecules in and out of the cell. (wikipedia.org)
- C8 alpha-gamma induces the polymerization of 10-16 molecules of C9 into a pore-forming structure known as the membrane attack complex. (wikipedia.org)
- C9 is one member of the complement membrane attack complex (MAC), which also includes complement components C5b, C6, C7 and C8. (wikipedia.org)
- Pore formation involves binding the C9 molecules to the target membrane, membrane molecules forming a pre-pore shape, and conformational change in the TMH1, the first transmembrane region, and TMH2, the second transmembrane region. (wikipedia.org)
- Protection of hDAF-transgenic porcine endothelial cells against activation by human complement: role of the membrane attack complex, Xenotransplantation, 9:97-105 (2002). (freepatentsonline.com)
- Complement in acute and chronic arthritides: assessment of C3c, C9 and protectin (CD59) in synovial membrane, Ann. (freepatentsonline.com)
- The complement system is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promotes inflammation, and attacks the pathogen's cell membrane . (wikipedia.org)
- The end result of this complement activation or complement fixation cascade is stimulation of phagocytes to clear foreign and damaged material, inflammation to attract additional phagocytes, and activation of the cell-killing membrane attack complex . (wikipedia.org)
- The immune complex serves as an activator that triggers a response from the C5b - C9 complements, which form a membrane attack complex (MAC) on the glomerular epithelial cells. (wikipedia.org)
- Immunofluorescence microscopy will reveal typical granular deposition of immunoglobulins and complement along the basement membrane. (wikipedia.org)
- The cleavage product C5B joins C6, C7, C8 and multiple copies of C9 to form the Membrane Attack Complex, which forms a channel for water to flood into the target cell, leading to osmotic lysis. (wikipathways.org)
- Blaas P, Berger B, Weber S, Peter HH, Hansch GM (1988) Paroxysmal nocturnal hemoglobinuria: enhanced stimulation of platelets by terminal complement components is related to the lack of C8bp in the membrane. (springer.com)
- Activation of C5 by a C5 convertase initiates the spontaneous assembly of the late complement components, C5-C9, into the membrane attack complex. (medindia.net)
- The former one was likely resulted from increased surface expression of HSP70 and complement oponozation ( 5 ), the latter one from membrane lipid alteration and cell apoptosis ( 6 ). (medsci.org)
Serum11
- Complement C9, Human, CAS 80295-59-6, is a native, single-chain glycoprotein present in normal human serum at 60 µg/ml. (emdmillipore.com)
- The total complement (CH50) assay (COM / Complement, Total, Serum) should be used as a screen for suspected complement deficiencies before ordering individual complement component assays. (testcatalog.org)
- The C5b-9 monomer having the sedimentation coefficient of 23S was extracted from the rabbit erythrocyte membranes that had been treated with a limiting amount of C9-deficient human serum and of 125 I-C9. (elsevier.com)
- This ELISA kit applies to the in vitro quantitative determination of Rat C9 concentrations in serum, plasma and other biological fluids. (srgroupchemical.com)
- Here, we charted in detail the structural microheterogeneity of C9 purified from human blood serum, using an integrative workflow combining high-resolution native mass spectrometry and (glyco)-peptide-centric proteomics. (figshare.com)
- The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 micrograms/ml in each of their 14 neonates. (nih.gov)
- Peerschke EI, Yin W, Alpert DR, Roubey RA, Salmon JE, Ghebrehiwet B. Serum complement activation on heterologous platelets is associated with arterial thrombosis in patients with systemic lupus erythematosus and antiphospholipid antibodies. (medscape.com)
- Therefore, our findings reveal that pathogen mounts a metabolic trick to cope with the serum complement-mediated killing. (frontiersin.org)
- We show here that while detectable levels of serum mAbs result in complement-mediated tumor cell lysis in vitro and tumor elimination in vivo , lower levels of antibody result in sublytic complement activation and more rapid tumor cell growth in vitro and in vivo . (aacrjournals.org)
- Serum-resistant strains of Borrelia burgdorferi evade complement-mediated killing by expressing a CD59-like complement inhibitory molecule. (nih.gov)
- Inhibition of the C8/C9 steps of complement lysis by a high density lipoprotein (HDL) of human serum. (yale.edu)
Deficiencies13
- Grumach AS, Kirschfink M. Are complement deficiencies really rare? (medscape.com)
- Complement deficiencies in systemic lupus erythematosus. (medscape.com)
- Complement genetics, deficiencies, and disease associations. (medscape.com)
- Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. (medscape.com)
- Complement deficiencies are said to comprise between 1 and 10% of all primary immunodeficiencies. (medscape.com)
- [ 3 ] A registry of complement deficiencies has been established as a means to promote joint projects on treatment and prevention of diseases associated with defective complement function. (medscape.com)
- This article outlines some of the disease states associated with complement deficiencies and their clinical implications. (medscape.com)
- Complement pathways and deficiencies. (medscape.com)
- Deficiencies in complement predispose patients to infection via 2 mechanisms: (1) ineffective opsonization and (2) defects in lytic activity (defects in MAC). (medscape.com)
- Specific complement deficiencies are also associated with an increased risk of developing autoimmune disease, such as SLE. (medscape.com)
- Complement tests, most commonly C3 and C4, are used to determine whether deficiencies or abnormalities in the complement system are causing, or contributing to, a person's disease or condition. (labcorp.com)
- Other complement components are ordered as needed to look for deficiencies. (labcorp.com)
- Ram S. Complement and deficiencies. (medlineplus.gov)
Antibody23
- The following antibody was used in this experiment: Complement C9 Polyclonal Antibody from Thermo Fisher Scientific, catalog # PA5-19826, RRID AB_10979448. (thermofisher.com)
- Western blot (WB) analysis of C9 antibody (Cat. (acris-antibodies.com)
- Western blot analysis of extracts from COLO cells, using C9 Antibody. (acris-antibodies.com)
- Immunofluorescence analysis of methanol-fixed HepG2, using Complement C9 antibody at 1/200 dilution. (acris-antibodies.com)
- C9 antibody diluted at 1:500. (acris-antibodies.com)
- Western blot analysis of extracts from COLO cells, using C9 antibody.The lane on the right is treated with the synthesized peptide. (acris-antibodies.com)
- Complement C9 antibody LS-C693053 is an FITC-conjugated guinea pig polyclonal antibody to rabbit Complement C9. (lsbio.com)
- Complement C9 antibody LS-C710425 is an HRP-conjugated rabbit polyclonal antibody to human Complement C9. (lsbio.com)
- Complement C9 antibody was raised against recombinant C9 (Gln22-His265) expressed in E. coli. (lsbio.com)
- The production of a monoclonal antibody that specifically targets the C9 binding site is necessary for further studies of Ts -Pmy function and may be used as a therapeutic agent for T. spiralis infection. (biomedcentral.com)
- In this study, a monoclonal antibody against the complement C9 binding domain of Ts -Pmy (mAb 9G3) was produced using hybridoma technology. (biomedcentral.com)
- mAb 9G3 is a specific antibody that binds to the C9 binding domain of Ts -Pmy and interferes with Ts -Pmy's complement-binding activity. (biomedcentral.com)
- The R-PLEX Human Complement C9 Antibody Set includes a matched capture and detection antibody pair and calibrator for the quick and easy measurement of Complement C9 in biological samples. (mesoscale.com)
- Calibration curve for the R-PLEX Human Complement C9 Antibody Set was assessed and representative data is presented below. (mesoscale.com)
- The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to Rat C9. (srgroupchemical.com)
- Then a biotinylated detection antibody specific for Rat C9 and Avidin-Horseradish Peroxidase (HRP) conjugate are added successively to each micro plate well and incubated. (srgroupchemical.com)
- Only those wells that contain Rat C9, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. (srgroupchemical.com)
- Lectins activate the lectin pathway in a manner similar to the antibody interaction with complement in the classical pathway. (medscape.com)
- Pharmacology and Biological Efficacy of a Recombinant, Humanized, Single-Chain Antibody CS Complement Inhibitor in Patients Undergoing Coronary Artery Bypass Graft Surgery With Cardiopulmonary Bypass, Circulation, 100:2499-2506 (1999). (freepatentsonline.com)
- The classical complement pathway typically requires antigen-antibody complexes for activation (specific immune response), whereas the alternative pathway can be activated by spontaneous C3 hydrolysis, foreign material, pathogens, or damaged cells. (wikipedia.org)
- The pathway by which antigen-antibody complex activates the complement system. (thefreedictionary.com)
- Therapy with tumor vaccines or complement-fixing monoclonal antibodies (mAb) inevitably results in sublytic levels of complement activation due to low antigen expression or periods of low antibody titers. (aacrjournals.org)
- Facilitation of complement-dependent killing of the Lyme disease spirochete, Borrelia burgdorferi, by specific immunoglobulin G Fab antibody fragments. (nih.gov)
Pathway15
- A rare defect of the complement classical pathway associated with susceptibility to severe recurrent infections, predominantly by Neisseria gonorrhoeae or Neisseria meningitidis. (abcam.com)
- The complement cascade consists of 3 separate pathways that converge in a final common pathway. (medscape.com)
- Among its related pathways are Complement and coagulation cascades and Immune response Lectin induced complement pathway . (genecards.org)
- Total complement activity (CH50 or CH100) may be ordered to look at the integrity of the entire classical complement pathway. (labcorp.com)
- Recently, properdin, a positive regulator of the alternative pathway of complement, has been shown to bind to surfaces and promote complement activation. (pubmedcentralcanada.ca)
- Moreover, the mechanisms involved in alternative pathway-mediated complement activation or its consequences on infection in C. pneumoniae remain unknown. (pubmedcentralcanada.ca)
- Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the lectin pathway . (wikipedia.org)
- While these two pathways differ from the classical pathway in their initial activation, all three complement pathways converge to similar outcomes. (thefreedictionary.com)
- The complement-activating polysaccharides found in medicinal plants are grouped into pectic polysaccharides (pectins, pectic arabinogalactans, and pectic herteroglycans), arabinogalactans, arabinans and other types such as mucilage, and it is suggested that they activate the complement system mainly through the classical pathway (Yamada and Kiyohara, 1999). (thefreedictionary.com)
- Individual chapter topics include the building blocks of the complement system, the classical pathway C1 complex, structure-function relationships in CD59, and cyclic antagonists of human C5a receptors. (thefreedictionary.com)
- Götze, O., Müller-Eberhard, H.-J.: The C3-activator system: an alternate pathway of complement activation. (springer.com)
- Complement-mediated accelerated growth of cultured tumor cell lines initiated by low mAb levels was associated with activation of the phosphoinositide 3-kinase (PI3K)/AKT survival pathway and significantly elevated levels of both p-AKT and p-PRAS40. (aacrjournals.org)
- This sublytic complement activation was associated with activation of the phosphoinositide 3-kinase (PI3K)/AKT survival pathway. (aacrjournals.org)
- V. IgG and F(ab')2 mediate killing of E. coli 0111B4 by the alternative complement pathway without increasing C5b-9 deposition. (nih.gov)
- Kenny DM, Davis AE (1981) Association of alternative complement pathway components with human blood platelets: secretion and localization of factor D and beta-1H globulin. (springer.com)
Late-acting complement com2
- generation of biologically active materials from late acting complement components and 2. (springer.com)
- It was also shown that the activation of the late-acting complement components (C3-C9) by endotoxin increased by 100 times after adrenalectomy in rats. (springer.com)
Cascade8
- Gene ID: 735) is the final step in the Complement cascade. (mesoscale.com)
- New studies point to the complex interplay between the complement cascade and adaptive immune response, and complement is also being studied in association with ischemic injury as a target of therapy. (medscape.com)
- The Classical Complement Cascade Mediates CNS Synapse Elimination. (freepatentsonline.com)
- The "complement cascade" is a series of reactions that take place in the blood. (medlineplus.gov)
- Bioinformatics analyses indicated that DEPs in both the EOPE and LOPE groups were associated with abnormalities in the activation of the coagulation cascade and complement system as well as with lipid metabolism. (peerj.com)
- The pathogenesis of EOPE and LOPE appeared to be associated with coagulation cascade activation, lipid metabolism, and complement activation. (peerj.com)
- The complement system is a biochemical cascade that helps, or complements, the ability of antibodies to clear pathogens from an organism. (wikipathways.org)
- Terminal complement components comprise the final 5 components of the complement cascade (C5 to C9). (scielo.org.za)
Molecules9
- After binding of C8, a variable number of C9 molecules associate with the C5b678 complex, which is also termed the terminal complement complex (TCC). (acris-antibodies.com)
- Full lytic activity of MAC occurs only after binding of three to six C9 molecules to each C5b-8 complex. (emdmillipore.com)
- Therefore, these results indicate that, although the binding of C9 molecules to the C5b-8 complex (C5b-9 monomer formation) does not significantly affect the susceptibility to proteases of the C9 molecules, C5b-9 dimer formation markedly limits the accessibility of proteases to the C9 subunit molecules. (elsevier.com)
- Active MAC is composed of the subunits C5b, C6, C7, C8 and several C9 molecules. (wikipedia.org)
- Similar hydrophobic sites on C8 and C9 molecules are exposed when they bind to the complex, so they can also insert into the bilayer. (wikipedia.org)
- Multiple molecules of C9 can join spontaneously in concentrated solution to form polymers of C9. (wikipedia.org)
- Once activated, about 12-18 molecules of C9 polymerize to form pores in target cell membranes, causing lysis and cell death. (wikipedia.org)
- The final step of MAC on target cell surfaces involves the polymerization of C9 molecules bound to C5b8 forming C5b-9. (wikipedia.org)
- C9 molecules allow cylindrical, asymmetrical transmembrane pores to form. (wikipedia.org)
Inflammation5
- at the same time, the inflammation promoted by complement activation can result in cellular damage when not kept in check. (medscape.com)
- Complement testing may be ordered when a person has unexplained inflammation or edema or symptoms of an autoimmune disorder such as SLE. (labcorp.com)
- Bean KV, Massey D, Gupta G. Mediators of inflammation: complement. (medlineplus.gov)
- Effects of Complement Inhibition with Soluble Complement Receptor-1 on Vascular Injury and Inflammation during Renal Allograft Rejection in the Rat, Am. J. Pathol. (freepatentsonline.com)
- Mediators of inflammation: complement. (ucsfhealth.org)
Deposition3
- Deposition of complement C9 in primary sclerosing cholangitis (PSC). (ox.ac.uk)
- Supplemental IgG enhanced 125I-labeled C9 deposition and potentiated the bactericidal activity of exogenous C9. (nih.gov)
- The binding of these physiological forms accelerates complement activation on the Chlamydia pneumoniae surface, as measured by C3b and C9 deposition. (pubmedcentralcanada.ca)
Immunology2
- Yamamoto, K & Migita, S 1981, ' Proteolysis of the monomeric and dimeric C5b-9 complexes of complement: Alteration in the susceptibility to proteases of the C9 subunits associated with C5b-9 dimerization ', Journal of Immunology , vol. 127, no. 2, pp. 423-426. (elsevier.com)
- In: Lambris JD (Current topics in microbiology and immunology, Vol 153) (ed) The third component of complement. (springer.com)
Perforin2
- Perforin has structural and functional similarities to complement component 9 (C9). (wikipedia.org)
- It was originally identified and named as being common to five complement components (C6, C7, C8-alpha, C8-beta, and C9) and perforin. (embl.de)
Lectin2
- All three pathways of the complement system ( classical , lectin and alternative pathways) initiate the formation of MAC. (wikipedia.org)
- The complement system is a central component of the innate immune response and is involved in many functions, including recognition, opsonization, phagocytosis, and destruction of foreign cells, as well as generation of chemotactic fragments (C3a and C5a) and activation of adaptive immunity ( 30 , 32 , 51 ) Three pathways of complement activation are known: the classical, lectin, and alternative pathways. (pubmedcentralcanada.ca)
Paroxysmal nocturnal hemoglobinuria with1
- Devine DV, Siegel RS, Rosse WF (1987) Interactions of the platelets in paroxysmal nocturnal hemoglobinuria with complement. (springer.com)
Susceptibility1
- [ 2 ] , whereas C5 to C9 may have enhanced susceptibility to meningococcal disease. (medscape.com)
Lysis1
- Activation of the complement system leads to the formation of the MAC, which in turn causes lysis, and death of target cells. (adipogen.com)
Activation13
- The three distinct activation pathways of complement converge with the formation of a C5 convertase. (acris-antibodies.com)
- TCC is present in normal human plasma and increased in patients with complement activation. (acris-antibodies.com)
- Human CD59 expressed in transgenic mouse hearts inhibits the activation of complement, 3:305-312 (1995). (freepatentsonline.com)
- Low molecular weight dextran sulfate prevents complement activation and delays hyperacute rejection in pig-to-human xenotransplantation models, Xenotransplantation, 8:24-35 (2001). (freepatentsonline.com)
- Effects of Complement Activation in the Isolated Heart, Circulation Research, 71:303-319 (1992). (freepatentsonline.com)
- Complement activation in stored platelet concentrates, Transfusion, 33:150-154 (1993). (freepatentsonline.com)
- Inflammatory cellular mediators, activation of the complement, by-products of coagulation and hemolysis such as thrombin and fibrin, and hemoglobin enter the brain and induce a local and systemic inflammatory reaction. (frontiersin.org)
- Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT. Complement system part I - molecular mechanisms of activation and regulation. (ucsfhealth.org)
- Activation of complement represents one means of natural resistance to infection from a wide variety of potential pathogens. (pubmedcentralcanada.ca)
- Here we studied whether properdin-mediated complement activation occurs on the surface of Chlamydia pneumoniae , an obligate intracellular Gram-negative bacterium that causes 10 to 20% of community-acquired pneumonia. (pubmedcentralcanada.ca)
- This complement-mediated PI3K activation and accelerated tumor growth in vitro and in vivo are eliminated by PI3K inhibitors NVP-BEZ235 and Wortmannin. (aacrjournals.org)
- Opportunities for new therapies based on the natural regulators of complement activation. (wikipathways.org)
- The increasing number of clinical conditions and pathologies that involve the complement system has generated significant interest in the development of therapeutic options to inhibit complement activation, which holds great promise in treating a range of diseases. (salesandmarketingnetwork.com)
Components9
- Complement components C6, C7, C8 and C9. (ebi.ac.uk)
- Complement C1r/C1s components (1 copy). (yale.edu)
- In fish, the liver is the site where the majority of complement components are produced and expressed, but C9 can also be found in other tissues. (wikipedia.org)
- MAC formation starts with the assembly of a tetrameric complex with the complement components C6, C7, C8, and C5b. (wikipedia.org)
- Individual complement components may be ordered when the total complement activity (CH50, sometimes called CH100) is abnormal to help determine which of the components are deficient or abnormal. (labcorp.com)
- C3 and C4 are the complement components measured most often. (medlineplus.gov)
- Betz M, Seitz M, Hansch GM (1987) Thromboxane B2 synthesis in human platelets induced by the late complement components C5b-9. (springer.com)
- Hansch GM, Gemsa D, Resch K (1985) Induction of prostanoid synthesis in human platelets by the late complement components C5b-9 and channel forming antibiotic nystatin: inhibition of the reacylation of liberated arachidonic acid. (springer.com)
- The roles of some of the components of the innate immune system have been tested in OA models in vivo including the roles of synovial macrophages and the complement system. (jrheum.org)
Reactivity2
- PA5-19826 targets C9 in IHC (P) and WB applications and shows reactivity with Human samples. (thermofisher.com)
- No significant cross-reactivity or interference between Rat C9 and analogues was observed. (srgroupchemical.com)
Functional2
- Also, because C9 is required for the MAC of complement to become functional, interfering with signaling pathways that stimulate its production could offer new therapeutic strategies for treating various neurodegenerative disorders. (adipogen.com)
- Functional complement C1q abnormality leads to impaired immune complexes and apoptotic cell clearance. (medscape.com)
Component of the complement1
- This gene encodes the final component of the complement system. (thermofisher.com)
Human C93
- Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human C9. (thermofisher.com)
- Native, human C9 complement component. (emdmillipore.com)
- Orthologous to human C9 (complement C9). (zfin.org)
Antibodies4
- The complement system can be recruited and brought into action by antibodies generated by the adaptive immune system . (wikipedia.org)
- In the early 20th century, this controversy was resolved when it became understood that complement can act in combination with specific antibodies, or on its own in a non-specific way. (wikipedia.org)
- These findings were mirrored by in vitro studies with antibodies against these antigens as well as GD2 and CD20 (with Rituxan), and shown to be complement-dependent in all cases. (aacrjournals.org)
- Antibodies specific to surface antigens are not effective in complement-mediated killing of Haemophilus ducreyi. (nih.gov)
Innate5
- The complement system is part of the innate immune system. (medscape.com)
- Although the complement system is part of the body's innate, relatively nonspecific defense against pathogens, its role is hardly primitive or easily understood. (medscape.com)
- 2001). "The complement system and innate immunity" . (wikipedia.org)
- During this journey, from circulating cells to endothelial cells, C. pneumoniae encounters the innate immune system, in which the complement system may play a fundamental role in controlling Chlamydia infection. (pubmedcentralcanada.ca)
- secondly, we review the literature that addresses the innate immune system, including the complement system and synovial macrophages, in the pathogenesis of OA. (jrheum.org)
Pathogens1
- Evasion of complement-mediated killing is a common phenotype for many different types of pathogens, but the mechanism is still poorly understood. (frontiersin.org)
System19
- Degn SE, Jensenius JC, Thiel S. Disease-causing mutations in genes of the complement system. (medscape.com)
- The complement system plays an important part in defense against pyogenic organisms. (medscape.com)
- In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). (medscape.com)
- These findings underscore the duality of the complement system. (medscape.com)
- Knowledge about the complement system is expanding. (medscape.com)
- An intricate system regulates complement activity. (medscape.com)
- Reid K. B. M., The complement system , in: B. D. Hames and D. M. Glover (eds. (wikipedia.org)
- Atkinson JP, Du Clos TW, Mold C, Kulkarni H, Hourcade D, Wu X. The human complement system: basic concepts and clinical relevance. (medlineplus.gov)
- It may also be ordered when a health practitioner suspects that someone may have an immune complex-related condition and wants to check the status of the person's complement system. (labcorp.com)
- Total complement activity (CH50, CH100) looks at the overall activity of the complement system. (medlineplus.gov)
- The complement system. (medlineplus.gov)
- Characterization of Complement Anaphylatoxins and Their Biological Responses, In: The Human Complement System in Health and Disease, Volanakis, J.E., Frank, M.M. (Eds. (freepatentsonline.com)
- Merle NS, Noe R, Halbwachs-Mecarelli L, Fremeaux-Bacchi V, Roumenina LT. Complement system part II: role in immunity. (ucsfhealth.org)
- Ehrlich introduced the term "complement" as part of his larger theory of the immune system. (wikipedia.org)
- Ehrlich, therefore, named this heat-labile component "complement," because it is something in the blood that "complements" the cells of the immune system. (wikipedia.org)
- When this happens, the complement system is activated and works as a cascading event like the falling of a row of dominoes. (lifeextension.com)
- This blood test measures component 3 (C3) of the complement system. (lifeextension.com)
- The interaction of blood platelets with the complement system is the subject of this review. (springer.com)
- Blajchman MA, Ozge-Anwar AH (1986) The role of the complement system in hemostasis. (springer.com)
Concentration3
- We measured the concentration of C9 in the CSF and plasma of 93 consecutive patients referred for CSF examination in an outpatient multispecialty clinic. (elsevier.com)
- The OD value is proportional to the concentration of Rat C9. (srgroupchemical.com)
- You can calculate the concentration of Rat C9 in the samples by comparing the OD of the samples to the standard curve. (srgroupchemical.com)
Genes2
- Expression of Complement Regulator Genes in Aβ1-42 Stimulated Human Neuroblastoma Cell. (freepatentsonline.com)
- C9 genes include 11 exons and 10 introns when found in fish. (wikipedia.org)
Levels of complement1
- Whereas uEVs of young patients with ADPKD and preserved kidney function already had higher levels of complement, only uEVs of patients with advanced stages of ADPKD had increased levels of villin-1, periplakin, and envoplakin. (asnjournals.org)
Species2
- Our Complement Component C9 Lysates can be used in a variety of model species. (novusbio.com)
- Role of a disulfide-bonded peptide loop within human complement C9 in the species-selectivity of complement inhibitor CD59. (embl.de)
Molecular1
- It resembled the previously described polymerized C9 (poly C9) produced from isolated monomeric C9 by prolonged incubation at 37° C in that it was resistant to dissociation by SDS and reducing agents and had an apparent molecular weight of ~1.1 million. (elsevier.com)
Porcine1
- Combined Inhibition of Apoptosis and Complement Improves Neural Graft Survival of Embryonic Rat and Porcine Mesencephalon in the Rat Brain, Exp. (freepatentsonline.com)
Total complement1
- This article describes the test that measures total complement activity. (medlineplus.gov)
MACPF1
- C6-C9 all contain a common MACPF domain. (wikipedia.org)
Diminished third component of complement1
- Leiner's disease associated with diminished third component of complement. (medscape.com)
Inhibitor2
- Transgenic Expression of a Soluble Complement Inhibitor Protects Against Renal Disease and Promotes Survival in MAU/pr Mice, J. Immunol. (freepatentsonline.com)
- A recombinant soluble chimeric complement inhibitor composed of human CD46 and COSS reduces acute cardiac tissue injury in models of pig-to-human heart transplantation, Transplantation, 69:2282-2289 (2000). (freepatentsonline.com)
Immunohistochemical2
- Immunohistochemical analysis of Complement C9 staining in human liver cancer formalin fixed paraffin embedded tissue section. (acris-antibodies.com)
- Human myocardium: immunohistochemical staining for complement component C9 using NCL-CCC9. (leicabiosystems.com)
Immune3
- Suppression of the Immune Response by a Soluble Complement Receptor of B Lymphocytes, 254:102-105 (1991). (freepatentsonline.com)
- Borsos, T., Rapp, H. J.: Immune hemolysis: A simplified method for the preparation of EAC'4 with guinea pig or with human complement. (springer.com)
- In addition, the combination of our transcriptomic data with an analysis of plasmatic immune parameters revealed a stimulation of complement activity associated with an immunodeficiency in the fish fed VD, and different inflammatory status between the two half-sibfamilies. (biomedcentral.com)
Pore2
- Mapping all detected and validated sites of modifications on a structural model of C9, as present in the MAC, hints at their putative roles in pore formation or receptor interactions. (figshare.com)
- Pore formation by C9 is an important way that bacterial cells are killed during an infection, and the target cell is often covered in multiple MACs. (wikipedia.org)