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.
Immune complexes and complement hypercatabolism in patients with leprosy. (1/321)
The occurrence of immune complexes in the serum and the level of the C3 breakdown product C3d in the plasma from patients with leprosy were studied by quantitative methods and the results were compared in various forms of the disease. These studies were performed on sixty-two samples from twenty-six patients. The serum 125I-C1q binding activity was found to be increased by more than 2 s.d., as compared to the normal values, in most of the sera from patients with erythema nodosum leprosum (ENL) (80%) and uncomplicated lepromatous leprosy (82%), but also in the sera from patients with tuberculoid leprosy (58%). In vitro studies suggested that immune complexes involving mycobacterial antigens were present in leprosy sera. An increased C3d level (greater than 2s.d.) was also found in most of the plasma from patients with ENL (70%), but rarely in the plasma from patients with uncomplicated lepromatous leprosy (18%) and never in tuberculoid leprosy patients' plasma. The absence of a significant correlation between the 125I-C1q binding activity and the C3d level in leprosy patients may suggest that extravascular immune complexes are involved in the complement activation occurring in ENL. The quantitation of C3d in plasma may be of some practical interest in the early diagnosis of ENL complications of leprosy. (+info)Binding of soluble immune complexes to Raji lymphocytes. Role of receptors for complement components, C1q and C3-C3b. (2/321)
We have found that, although the binding of particulate antigen-antibody complement complexes such as EAC to lymphoblastoid Raji cells is mediated largely through receptors for C3b, the binding of complement-containing soluble complexes such as those prepared with aggregated human IgG (AHG) occurs also via receptors for C1q. Evidence supporting this conclusion included: (1) Binding of AHG to Raji cells takes place after incubation in EDTA serum; (2) Binding of AHG does not occur in C1q deficient EDTA serum but does take place after addition of C1q; (3) The extent of binding of AHG in EDTA serum is a function of the amount of C1q present; (4) Raji cells can bind up to 5-4 times 10(5) molecules of 125I C1q per cell which can be blocked by unlabelled C1q; (5) AHG pre-incubated with C can bind to a T-cell line MOLT, which lacks receptors for C3b but possesses receptors for C1q to the same extent as Raji cells; (6) Immunoassays for immune complexes in human sera yield similar results whether Raji cells, MOLT cells or C1q precipitation is used for assay; (7) EAC-Raji cell rosettes can be inhibited with inulin-treated, C1q deficient serum containing C3b or C3d whereas binding of AHG or immune complexes in patient samples to Raji or MOLT cells is not inhibited by this reagent. We conclude that receptors for C1q on certain B and T lymphocytes may play an important role in physiologic functions of lymphocytes depending on binding of soluble immune complexes to their surfaces. (+info)A truncated form of mannose-binding lectin-associated serine protease (MASP)-2 expressed by alternative polyadenylation is a component of the lectin complement pathway. (3/321)
The lectin complement pathway is initiated by binding of mannose-binding lectin (MBL) and MBL-associated serine protease (MASP) to carbohydrates. In the human lectin pathway, MASP-1 and MASP-2 are involved in the proteolysis of C4, C2 and C3. Here we report that the human MBL-MASP complex contains a new 22 kDa protein [small MBL-associated protein (sMAP)] bound to MASP-1. Analysis of the nucleotide sequence of sMAP cDNA revealed that it is a truncated form of MASP-2, consisting of the first two domains (i.e. the first internal repeat and the epidermal growth factor-like domain) with four different C-terminal amino acids. sMAP mRNAs are expressed in liver by alternative polyadenylation of the MASP-2 gene, in which a sMAP-specific exon containing an in-frame stop codon and a polyadenylation signal is used. The involvement of sMAP in the MBL-MASP complex suggests that the activation mechanism of the lectin pathway is more complicated than that of the classical pathway. (+info)Isolation of two forms of activated C1s, a subcomponent of the first component of rabbit complement. (4/321)
Two forms of activated C1s, a subcomponent of the first component of complement, were present in preparations of C1 specifically purified from rabbit serum by affinity chromatography on IgG-Sepharose 6B and were separated by DEAE-cellulose chromatography in the presence of EDTA. These two activated C1s, designated C1s(I) and C1s(II), were indistinguishable with regard to hemolytic activity as well as C1s esterase activity, though they had different molecular weights. C1s(I) had a molecular weight of 106,000, consisting of H and L chains connected by disulfide bonds; the molecular weights of the chains were 70,000 and 36,000, respectively. On the other hand, C1s(II), with a molecular weight of 72,000, consisted of two chains each with a molecular weight of about 37,000, which were also connected by disulfide bonds. These results suggest that, in the case of rabbit C1s, the primary product of activation with C1r, C1s(I), may be susceptible to further cleavage of its H chain without any loss of C1s activity, resulting in the formation of C1s(II), though the active principle responsible for this conversion remains to be elucidated. (+info)Complement C1s activation in degenerating articular cartilage of rheumatoid arthritis patients: immunohistochemical studies with an active form specific antibody. (5/321)
OBJECTIVE: The first complement component C1s was reported to have novel functions to degrade matrix components, besides its activities in the classic complement pathway. This study explores participation of C1s in articular cartilage degradation in rheumatoid arthritis (RA). METHODS: Normal articular cartilage (n = 6) and cartilage obtained from joints with RA (n = 15) and osteoarthritis (OA, n = 10) were immunostained using anti-C1s monoclonal antibodies PG11, which recognises both active and inactive C1s, and M241, which is specifically reactive to activated C1s. The effects of inflammatory cytokines on C1s production by human articular chondrocytes were also examined by sandwich ELISA. RESULTS: In normal articular cartilage, C1s was negative in staining with both PG11 and M241. In contrast, degenerating cartilage of RA was stained with PG11 (14 of 15 cases), and in most of the cases (13 of 15 cases) C1s was activated as revealed by M241 staining. In OA, C1s staining was restricted in severely degrading part of cartilage (5 of 10 cases), and even in that part C1s was not activated. In addition, C1s production by chondrocytes in vitro was increased by an inflammatory cytokine, tumour necrosis factor alpha. CONCLUSION: These results suggest that C1s activated in degenerative cartilage matrix of RA but not in that of OA. C1s is thought to participate in the pathogenesis of RA through its collagenolytic activity in addition to the role in the classic cascade. (+info)Possible mechanisms of the first step of the classical complement activation pathway: binding and activation of C1. (6/321)
Different immunoglobulin preparations of human monoclonal IgM, normal human and rat IgG, as well as purified rabbit antibodies were treated by various methods, fragmentation, aggregation and complexing with antigen. The ability of the treated and untreated preparations to fix isolated human C1, to activate the classical complement pathway (to consume C4 in normal human serum) were compared. It was found that the different methods affected the conformation of the immunoglobulin molecules in different ways and induced changes to a greater or lesser extent in the two capacities of the preparations tested. In the case of the monoclonal IgM preparation a strong C1-fixation was observed without measurable complement activation. Other preparations, interfacially aggregated human IgG, BSA-anti-BSA and OA-anti-OA immune complexes had a very weak C1-fixing but a marked complement activating capacity. Some preparations, e.g. heat-aggregated IgG, both fixed and activated C1 effectively, aggregates with a complement-activating capacity without C1-fixing effect were separated by gel-filtration. It was demonstrated further, that at a given time only a part of the activated C1 molecules could be found fixed to the immunoglobulins, the other part was released into the fluid phase after activation. On the basis of the results of this and previous studies a hypothesis is proposed suggesting three possible results of the interaction between C1 and the different preparations: (1) firm fixation and activation; (2) binding not followed by activation and (3) a transient binding leading to activation. The possible application of this hypothesis for the interpretation of the results of the different methods for detecting immune complexes is discussed. (+info)C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. (7/321)
Previously, we showed that soluble C1q bound specifically to CR1 on transfected cells. If the CR1-C1q interaction were to participate in immune complex clearance, then this interaction should support E adhesion. Using a tip plate adhesion assay, we found that immobilized C1q mediated adhesion of human E. E binding to C1q was specifically inhibited by polyclonal anti-CR1 Fab fragments. Intact C1 was not efficient as an adherence ligand until it was treated with EDTA or the C1 inhibitor to remove the C1r2C1s2 complex from C1, leaving C1q. Titration of C1q alone, C4b alone, and C1q + C4b indicated that the two complement ligands were additive in their ability to support CR1-mediated adhesion of E. Analysis of binding to immobilized CR1 using a BIAcore instrument documented that C1q, C4b, and C3b binding were independent events. Additionally, C1q-dependent binding of immune complexes and heat-aggregated IgG to E was documented. These experiments confirm that the immune adherence receptor in humans, CR1, is the single receptor for all of the opsonic ligands of complement, provide evidence for a single C1q binding site on LHR-D of CR1, and suggest that C1q may participate in immune clearance. (+info)Inhibitory effect of bilirubin on complement-mediated hemolysis. (8/321)
We investigated the in vitro action of the bile pigments, unconjugated bilirubin (UB) and bilirubin monoglucuronide (BMG) on complement (C) cascade reaction. Both UB and BMG inhibited hemolysis in the classical pathway (CP) in a dose-dependent manner at low micromolar concentrations, UB showing a stronger effect than BMG. The analysis of the action of UB on the hemolytic activity of the C1, C4, C2 and C-EDTA components of the C cascade revealed that the C1 step was the most inhibited. An enzyme immunoassay was developed to evaluate the effect of UB on the binding of C1q, one of the subcomponents of C1, to human IgM and IgG. The study demonstrated that the unconjugated pigment interferes both the C1q-IgM and -IgG interactions, thus tentatively explaining the inhibitory action of UB on hemolytic activity of C1. We conclude that the anti-complement effect of UB is mainly exerted on the C1 component, the recognition unit of CP. The potential clinical implication of the reported effects in hyperbilirubinemia is discussed. (+info)
A collagen-like amino acid sequence in a polypeptide chain of human C1q (a subcomponent of the first component of complement) |...
Interaction of the envelope clycoprotein of human immunodeficiency virus with C1Q and fibronectin under the low ionic strength...
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Ganeden
"Kerry Group acquires probiotic player Ganeden to complement existing portfolio". nutraingredients.com. Retrieved 2017-10-11. ... C1 Grant, Alison (June 6, 2011). "Ganeden sells probiotic brands to Schiff Nutrition International". Cleveland Plain Dealer. " ...
Sierpiński carpet
Suppose its complement in the plane has countably many connected components C1, C2, C3, ... and suppose: the diameter of Ci ...
GraalVM
It complements or replaces the existing compilers (C1/C2 in HotSpot). In contrast to those existing compilers, the GraalVM ... The GraalVM compiler was started by manually converting the code of the Hotspot client compiler (named "C1") into Java, ...
C3b
The C1 complement complex binds to these antibodies resulting in its activation via cross proteolysis. This activated C1 ... C3b is the larger of two elements formed by the cleavage of complement component 3, and is considered an important part of the ... The key to the success of the complement system in clearing antigens is regulating the effects of C3b to pathogens alone and ... C3a - the other fragment C3 is cleaved into along with C3b Liszewski, M. Kathryn; Atkinson, John P. (2015-06-10). "Complement ...
Angioedema
In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... C1-esterase (aka: C1-inhibitor or C1INH), and continuous production of kallikrein, another process inhibited by C1INH. This ...
Albert J. R. Heck
"Molecular Basis of Assembly and Activation of Complement Component C1 in Complex with Immunoglobulin G1 and Antigen". Molecular ... antibodies in complex with their antigens or proteins of the complement system. He has developed, in collaboration with ...
Angioedema
In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... Ruconest (C1-inhibitor). References[edit]. *^ a b c d e f g h i j k l m n o p q r s t Bernstein, JA; Cremonesi, P; Hoffmann, TK ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered ...
Lupus erythematosus
When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The ...
C1QBP
The human complement subcomponent C1q associates with C1r and C1s in order to yield the first component of the serum complement ... The protein encoded by this gene is known to bind to the globular heads of C1q molecules and inhibit C1 activation. This ... C1QBP, GHABP1, SF2p32, gC1Q-R, gC1qR, p32, complement component 1, q subcomponent binding protein, complement C1q binding ... Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene.[ ...
Polyclonal antibodies
Chicken IgY does not fix mammalian complement component C1 and it does not perform as a precipitating antibody using standard ... complement fixing nature] of the antibodies to be made. Immunization and phlebotomies are stress associated and, at least when ...
Aggrecan
... chondroitin sulfate proteoglycan of human B cell lines binds to the complement protein C1q and inhibits complex formation of C1 ... complement binding protein (CBP)-like domains, immunoglobulin folds and proteoglycan tandem repeats. Aggrecan is a critical ...
CSPG4
... chondroitin sulfate proteoglycan of human B cell lines binds to the complement protein C1q and inhibits complex formation of C1 ...
Borrelia burgdorferi
"Borrelia burgdorferi BBK32 Inhibits the Classical Pathway by Blocking Activation of the C1 Complement Complex", PLoS Pathogens ... Zipfel P., Hallström T., Riesbeck K. (2013). "Human complement control and complement evasion by pathogenic microbes - Tipping ... As a result, C1 is trapped in an inactive form. B. burgdorferi (B31 strain) was the third microbial genome ever sequenced, ... This is part of a complement system evasion strategy that leads to downstream blocking of immune response. In addition, ...
Basigin
10 C1,10 39.72 cM. Start. 79,704,491 bp[2]. End. 79,711,969 bp[2]. ...
Urticarial vasculitis
... named C1-inhibitor. The inhibition of C1-inhibitor leads to over-activation of the complement pathway and one protein that ... Consequently, levels of all complement proteins become low. The complement pathway is composed of several subset pathways: the ... Also, there has been some speculation as to an additional autoantibody against an inhibitor protein (in the complement pathway ... C1q is an integral component within the complement pathway - a complicated cascade of protein interactions, culminating in an ...
Procollagen-proline dioxygenase
Müller W, Hanauske-Abel H, Loos M (October 1978). "Biosynthesis of the first component of complement by human and guinea pig ... peritoneal macrophages: evidence for an independent production of the C1 subunits". Journal of Immunology. 121 (4): 1578-84. ...
C1QA
1992). "Human immunodeficiency virus type 1 activates the classical pathway of complement by direct C1 binding through specific ... 1994). "Regulation of the synthesis of C1 subcomponents and C1-inhibitor". Behring Inst. Mitt. (93): 196-203. PMID 8172568. ... Complement C1q subcomponent subunit A is a protein that in humans is encoded by the C1QA gene. This gene encodes a major ... "Entrez Gene: C1QA complement component 1, q subcomponent, A chain". Human C1QA genome location and C1QA gene details page in ...
Enhanced vegetation index
The coefficients adopted in the MODIS-EVI algorithm are; L=1, C1 = 6, C2 = 7.5, and G (gain factor) = 2.5. Whereas the ... This could potentially lead to a 30-year EVI record that complements the NDVI record. The blue band has always been problematic ... The two vegetation indices complement each other in global vegetation studies and improve upon the detection of vegetation ... and C1, C2 are the coefficients of the aerosol resistance term, which uses the blue band to correct for aerosol influences in ...
Immunoglobulin M
IgM interacts with several other physiological molecules: IgM can bind complement component C1 and activate the classical ... nor in mutant animals lacking complement receptors 1 and 2. Similarly, mutant IgM that cannot activate complement does not ... Several lines of evidence indicate that the ability of IgM to activate complement is required for its enhancing effect. That is ... Collins, C.; Tsui, F. W.; Shulman, M. J. (2002). "Differential activation of human and guinea pig complement by pentameric and ...
Centro Sismológico Nacional
In addition to the real-time system described above, 297 strong-motion offline instruments complement the network for ... under networks C and C1. Strong-motion data for recorded accelerations larger than are available through the CSN webpage. ...
Serpin
Beinrohr L, Harmat V, Dobó J, Lörincz Z, Gál P, Závodszky P (July 2007). "C1 inhibitor serpin domain structure reveals the ... Mollnes TE, Jokiranta TS, Truedsson L, Nilsson B, Rodriguez de Cordoba S, Kirschfink M (September 2007). "Complement analysis ... and C1-inhibitor) and tissue remodelling (PAI-1). By inhibiting signalling cascade proteases, they can also affect development ... related to deficiency of C1-inhibitor, and familial encephalopathy with neuroserpin inclusion bodies (FENIB; a rare type of ...
Astronomical catalog
Sir Patrick Moore compiled the Caldwell catalogue in 1995 to complement the Messier catalog, listing 109 bright star clusters, ... nebulae, and galaxies named C1 to C109. This is a list of deep-sky objects of interest to amateur astronomy and not a catalog ...
Domain (mathematical analysis)
A bounded domain is a domain which is a bounded set, while an exterior or external domain is the interior of the complement of ... C1 boundary, and so forth. ...
Xenotransplantation
... soluble complement receptor type 1, anti-C5 antibodies, or C1 inhibitor (C1-INH). Disadvantages of this approach include the ... The binding of XNAs initiate complement activation through the classical complement pathway. Complement activation causes a ... Interruption of the complement cascade *The recipient's complement cascade can be inhibited through the use of cobra venom ... Expression of human complement regulators (CD55, CD46, and CD59) to inhibit the complement cascade.[27] ...
Primary immunodeficiency
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C1-inhibitor deficiency (hereditary angioedema) Factor I deficiency (pyogenic infections) Factor H deficiency (haemolytic- ... 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 ...
Juvenile hormone diol kinase
Moreover, the hydrophobic nature of the binding pocket complements the C1 ester of the substrate and supports the observation ...
CUB domain
Mammalian complement subcomponents C1s/C1r, which form the calcium-dependent complex C1, the first component of the classical ... Mammalian complement-activating component of Ra-reactive factor (RARF), a protease that cleaves the C4 component of complement ... Almost all CUB domains contain four conserved cysteines that probably form two disulphide bridges (C1-C2, C3-C4). The CUB1 ... For example, in the complement proteases, the CUB domains mediate dimerisation and binding to collagen-like regions of target ...
Opsonin
The C1 complement complex can also interact with the Fc region of IgG and IgM immune complexes activating the classical ... Complement proteins involved in innate opsonization include C4b, C3b and iC3b. In the alternative pathway of complement ... C1q association eventually leads to the recruitment of complement C4b and C3b, both of which are recognized by complement ... Opsonins of the innate immune system largely comprise complement proteins, though interaction between complement proteins and ...
Mann-Whitney U test
displaystyle M={1 \over c(c-1)}\sum \mathrm {AUC} _{k,l}}. Where c is the number of classes, and the Rk,l term of AUCk,l ... Stated another way, the correlation is the difference between the common language effect size and its complement: r. =. f. −. ( ...
Chiropractic
"Evid Based Complement Alternat Med. 3 (4): 425-32. doi:10.1093/ecam/nel044. PMC 1697737 . PMID 17173105. Archived from the ... doi:10.1097/01.brs.0000167821.39373.c1. PMID 15990659.. *^ "Safety and regulation of chiropractic". NHS Choices. 20 August 2014 ... J Altern Complement Med. 14 (4): 361-68. doi:10.1089/acm.2007.0766. PMID 18435599.. ... J Altern Complement Med. 13 (5): 491-512. doi:10.1089/acm.2007.7088. PMID 17604553.. ...
T-72 operators and variants
The gunner still has the 1A40-4 FCS with 1K13-49 sight, but these are now part of the auxiliary sighting system to complement ... it is similar to that used in C1 Ariete) which enables a "hunter-killer" mode of operation; the commander has a panoramic day/ ...
Hyperimmunoglobulin E syndrome
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ...
Komplemendisüsteem - Vikipeedia
Klassikalist rada inhibeerib C1-inhibiitor, mis seostudes C1 valguga hoiab ära selle aktiveerumise. Alternatiivset rada ... Zipfel, P. F., Hallström, T., & Riesbeck, K. (2013). Human complement control and complement evasion by pathogenic microbes- ... 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. ...
Outline of brain mapping
The first negative and positive swings (see Visual N1, C1 and P1 (neuroscience)) in response to visual stimulation are of ... as the methods complement each other. Neuronal (MEG) and hemodynamic (fMRI) data do not necessarily agree, in spite of the ...
Vitamin C
This is followed by lactone formation with the hydrolase gluconolactonase between the carbonyl on C1 and hydroxyl group on C4. ... "Toward a phylogenetic classification of Primates based on DNA evidence complemented by fossil evidence". Molecular ... l-galactose dehydrogenase, whereby the lactone ring opens and forms again but with between the carbonyl on C1 and hydroxyl ...
Type C4-class ship
Complement:. Varied by design type. The Type C4-class ship were the largest cargo ships built by the United States Maritime ... Type C1 ship. *Type C2 ship. *Type C3 ship. *T2 tanker. *Liberty ship ...
Antibody-dependent cellular cytotoxicity
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... ADCC is independent of the immune complement system that also lyses targets but does not require any other cell. ADCC requires ... 1987). "Functional role of the alpha-chain of complement receptor type 3 in human eosinophil-dependent antibody-mediated ...
Hyderabad
"C-1 Population By Religious Community". Government of India, Ministry of Home Affairs. Archived from the original on 13 ... Complementing these government services are minibus routes operated by Setwin (Society for Employment Promotion & Training in ... which are complemented by private courier services.[46] ...
Torcetrapib
FC(F)(F)c1cc(cc(c1)C(F)(F)F)CN(C(=O)OC)[[email protected]@H]3c2c(ccc(c2)C(F)(F)F)N(C(=O)OCC)[[email protected]@H](C3)CC ... Drugs that interfere with the action of these peptides would aid in lowering cholesterol levels by complementing the action of ...
FOXA3
Corrocher R, Tedesco F, Rabusin P, De Sandre G (1975). "Effect of human erythrocyte stromata on complement activation". Br. J. ...
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 ... involved in the complement system. C1s is part of the C1 complex.[5][6][7][8] In humans, it is encoded by the C1S gene.[9] ... complement activation, classical pathway. • immune system process. • innate immune response. • ...
Adalimumab
CC(C)(C)C(=O)OC1=CC=C(C=C1)S(=O)(=O)NC1=CC=CC=C1C(=O)NCC(O)=O ... Complement component 5 *Eculizumab. *TNF *Adalimumab. * ...
Kolektin - Wikipedie
Watford WT, Ghio AJ, Wright JR (2000). "Complement-mediated host defense in the lung". Am J Physiol Lung Cell Mol Physiol 279: ... SP-A inhibuje rozpoznání imunitního komplexu C1q i C1.. Schopnost inhibovat klasickou cestu aktivace komplementu je specifická ... Watford WT, Wright JR, Hester CG, Jiang H, Frank MM (2001). "Surfactant Protein A Regulates Complement Activation". J Immunol ... Klasická cesta aktivace komplementu je spuštěna komplexem C1 (C1q, C1r, C1s), rozpozná-li Fc oblast imunoglobulinu navázaného ...
Complement system
Activation of complements by antigen-associated antibodyEdit. In the classical pathway, C1 binds with its C1q subunits to Fc ... 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 ...
Immunodeficiency
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... Complement deficiency is where the function of the complement system is deficient ...
ANSI escape code
Fe (C1 set element) ANSI escape sequences (not an exhaustive list) C1. Abbr. Name. Effect ... The change was designed to complement the Windows Subsystem for Linux, adding to the Windows Console Host used by Command ... Delegated to the applicable C1 control code standard.[21]:13.2.1 Accordingly, all escape sequences corresponding to C1 control ... which would form C1 codes.[clarification needed]. *Konsole generates SS3 modifiers char. when F1-F4 are pressed with modifiers. ...
Trehalose
"A Selaginella lepidophylla trehalose-6-phosphate synthase complements growth and stress-tolerance defects in a yeast tps1 ... 68.9 g per 100 g at 20 °C[1]. Solubility soluble in ethanol, insoluble in diethyl ether and benzene[2]. ...
Complement membrane attack complex
Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ... Reid K. B. M., The complement system, in: B. D. Hames and D. M. Glover (eds.), Molecular Immunology, Oxford: IRL Press, 1988, ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ...
Pitch class
... then C♯ = 1 ... A♯ = 10, B = 11, with "10" and "11" substituted by "t" and "e" in some sources,[5] A and B in others[6] (like ...
Dynein
Proper segregation is essential for producing haploid meiotic products with a normal complement of chromosomes. The formation ...
The Real Adventures of Jonny Quest
Complementing the show's educational, real-world premise, the site hosted links to academic, archaeological, and exploratory ... Peoria Journal Star: C1.. .mw-parser-output cite.citation{font-style:inherit}.mw-parser-output .citation q{quotes ...
Ciclosporin
InChI=1S/C62H111N11O12/c1-25-27-28-40(15)52(75)51-56(79)65-43(26-2)58(81)67(18)33-48(74)68(19)44(29-34(3)4)55(78)66-49(38(11)12 ...
XYY syndrome
p. C1. An Australian murder case that was reported to have been decided on the basis of the so-called XYY syndrome actually was ... Erring in the other direction, however, is the XYY complement resulting in the "supermale." He is also unusually tall and ... Court Brown; W. Michael (December 1968). "Males with an XYY sex chromosome complement". J Med Genet. 5 (4): 341-59. doi:10.1136 ... the sex chromosome complement is XXY rather than XY, thus erring in the direction of femaleness. The resulting individual, ...
Protease
C1-inhibitor (which protects the body from excessive protease-triggered activation of its own complement system), antithrombin ... Here, it is released by mast cells and causes activation of complement and kinins.[5] This group includes the calpains. ... the complement system, apoptosis pathways, and the invertebrate prophenoloxidase-activating cascade). Proteases can either ...
120220: Complement C1 Esterase Inhibitor, Functional | LabCorp
LabCorp test details for Complement C1 Esterase Inhibitor, Functional ... C1-inhibitor deficiency and angioedema. In Whaley K, ed. Complement in Health and Disease. MTP Press Limited; 1979:53. ... C1 Esterase Inhibitor, Func. 48494-9. 120221. C1 Est.Inhib.Funct.. %mean normal. 48494-9. ... and C1r subcomponent of the first component of complement by serum C1 esterase inhibitor. J Exp Med. 1969; 129(2):315-331. ...
complement C1 esterase inhibitor - WellSpan Health Library
Complement C1 esterase inhibitor is a man-made form of a protein in blood that helps control swelling in the body. People with ... What is complement C1 esterase inhibitor?. Complement C1 esterase inhibitor is a man-made form of a protein in blood that helps ... Complement C1 esterase inhibitor is used in people with hereditary angioedema. Berinert is used to treat attacks of angioedema ... What are the possible side effects of complement C1 esterase inhibitor?. Get emergency medical help if you have signs of an ...
Complement C1 Inhibitor Protein | Harvard Catalyst Profiles | Harvard Catalyst
Complement C1 Inactivator Proteins [D12.776.124.486.274.920.250]. *Complement C1 Inhibitor Protein [D12.776.124.486.274.920. ... "Complement C1 Inhibitor Protein" by people in Harvard Catalyst Profiles by year, and whether "Complement C1 Inhibitor Protein" ... "Complement C1 Inhibitor Protein" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH ( ... including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING ...
Peptide Inhibitor of Complement C1 (PIC1) Rapidly Inhibits Complement Activation after Intravascular Injection in Rats
Consequently, therapeutic modulators of the classical, lectin and alternative pathways of the complement system are currently ... The complement system has been increasingly recognized to play a pivotal role in a variety of inflammatory and autoimmune ... Peptide Inhibitor of Complement C1 (PIC1) Rapidly Inhibits Complement Activation after Intravascular Injection in Rats PLoS One ... specifically inhibits the classical and lectin pathways of complement and is referred to as Peptide Inhibitor of Complement C1 ...
complement C1 inhibitor protein | American Society of Nephrology
Final Report Summary - COMPLEMENT AND LDL (Complement and atherosclerosis: molecular mechanism of C1 activation by...
E-LDL is recognised by the C1q subunit of C1 and triggers direct C1 activation, suggesting that complement activation by E-LDL ... Final Report Summary - COMPLEMENT AND LDL (Complement and atherosclerosis: molecular mechanism of C1 activation by ... COMPLEMENT AND ATHEROSCLEROSIS: MOLECULAR MECHANISM OF C1 ACTIVATION BY ENZYMATICALLY MODIFIED LOW-DENSITY LIPOPROTEINS. ... to activate C1, the complex that triggers activation of the classical pathway of complement. ...
The First Component of Human Complement (C1): Kinetics of Reaction with its Natural Substrates | The Journal of Immunology
C1) of human complement. The data indicated that the increased rate of cleavage of C2 by C1 in the presence of C4 was due to ... The First Component of Human Complement (C1): Kinetics of Reaction with its Natural Substrates. Robert Strunk and Harvey R. ... The First Component of Human Complement (C1): Kinetics of Reaction with its Natural Substrates ... The First Component of Human Complement (C1): Kinetics of Reaction with its Natural Substrates ...
Classical Pathway of Complement Activation: Longitudinal Associations of C1q and C1-INH With Cardiovascular Outcomes |...
Classical Pathway of Complement Activation: Longitudinal Associations of C1q and C1-INH With Cardiovascular Outcomes. The CODAM ... Classical Pathway of Complement Activation: Longitudinal Associations of C1q and C1-INH With Cardiovascular Outcomes ... Classical Pathway of Complement Activation: Longitudinal Associations of C1q and C1-INH With Cardiovascular Outcomes ... Classical Pathway of Complement Activation: Longitudinal Associations of C1q and C1-INH With Cardiovascular Outcomes ...
BrkA Protein of Bordetella pertussisInhibits the Classical Pathway of Complement after C1 Deposition | Infection and Immunity
Deposition of purified C1. Bacteria were incubated with purified C1 (a and d), purified C1 with purified IgG (b and e), or heat ... C1 binding was investigated further. Incubation of B. pertussis with purified C1 alone, purified C1 with purified IgG, or heat- ... preventing complement function or recruiting a complement-inhibitory protein. BrkA could prevent complement function by acting ... BrkA Protein of Bordetella pertussisInhibits the Classical Pathway of Complement after C1 Deposition. Michael G. Barnes, Alison ...
CFH / Complement Factor H Antibody (clone C1, HRP) for WB/Western LS-C707907
Complement Factor H antibody LS-C707907 is an HRP-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ... About CFH / Complement Factor H. CFH / Complement Factor H is a member of the Regulator of Complement Activation (RCA) gene ... Complement Factor H antibody LS-C707907 is an HRP-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ... Complement Factor H antibody LS-C707907 is an HRP-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ...
CFH / Complement Factor H Antibody (clone C1, Cy3) for WB/Western LS-C698886
Complement Factor H antibody LS-C698886 is a Cy3-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ... About CFH / Complement Factor H. CFH / Complement Factor H is a member of the Regulator of Complement Activation (RCA) gene ... Complement Factor H antibody LS-C698886 is a Cy3-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ... Complement Factor H antibody LS-C698886 is a Cy3-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). ...
The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement |...
The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement. R B ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ... The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement ...
The catalytic chain of human complement subcomponent C1̅. Purification and N-terminal amino acid sequences of the major...
The catalytic chain of human complement subcomponent C1̅. Purification and N-terminal amino acid sequences of the major ... The catalytic chain of human complement subcomponent C1̅. Purification and N-terminal amino acid sequences of the major ... The catalytic chain of human complement subcomponent C1̅. Purification and N-terminal amino acid sequences of the major ... The catalytic chain of human complement subcomponent C1̅. Purification and N-terminal amino acid sequences of the major ...
Peptide inhibitor of complement C1 modulates acute intravascular hemolysis of mismatched red blood cells in rats<...
Peptide inhibitor of complement C1 modulates acute intravascular hemolysis of mismatched red blood cells in rats. Transfusion. ... Peptide inhibitor of complement C1 modulates acute intravascular hemolysis of mismatched red blood cells in rats. / Kumar, ... Peptide inhibitor of complement C1 modulates acute intravascular hemolysis of mismatched red blood cells in rats. ... The objective of this study was to evaluate the role of the classical pathway inhibitor peptide inhibitor of complement C1 ( ...
C1 | protein | Britannica.com
... complement protein in the pathway, C1, must interact with a bound immunoglobulin. Specifically, C1 interacts with the tail of ... Once bound to the antibody, C1 is cleaved, a process that activates… ... Other articles where C1 is discussed: immune system: Activation of the complement system: … ... complement protein in the pathway, C1, must interact with a bound immunoglobulin. Specifically, C1 interacts with the tail of ...
Complement c1 inactivator proteins facts | Factolex
C1-inhibitor) -- a serine protease inhibitor (serpin) protein, the main function of which is the inhibition of the complement ... Home » Index » C » Complement c1 inactivator proteins (C1-inhibitor) C1-inhibitor. also called Complement c1 inactivator ... This way, C1-inh prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. created by factobot ... irreversibly binds to and inactivates C1r and C1s proteinases in the C1 complex of classical pathway of complement. created by ...
JoVE Search Results: Complement C1 Inhibitor Protein
Complement, Immunity, Disease, Morbidity, Risk, Mortality, and Treatment ... Human complement regulators C4b-binding protein and C1 esterase inhibitor interact with a novel outer surface protein of ... Mechanisms of complement activation and effects of C1-inhibitor on the meconium-induced inflammatory reaction in human cord ... Human pasteurized C1-inhibitor concentrate for the treatment of hereditary angioedema due to C1-inhibitor deficiency. Abstract ...
ivd-test-reagent-kits-immunoassay-protein-complement-component-c1-inhibitor
Entry Terms : "C1 Inhibitor (Complement Component) Determination Reagents" , "Alpha-2-Globulin (C1 Protein Inhibitor) ... IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C1 Inhibitor. Definition : Immunoassay reagents intended to ... Home > Specialties > IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C1 Inhibitor ... an esterase inhibitor of the C1 protein found in the classic pathway of the complement components proteins. The absence of this ...
The complement system and innate immunity - Immunobiology - NCBI Bookshelf
Indeed, it now seems clear that complement first evolved as part of the innate immune system, where it still plays an important ... Although first discovered as an effector arm of the antibody response, complement can also be activated early in infection in ... This activity was said to complement the antibacterial activity of antibody, hence the name. ... Complement was discovered many years ago as a heat-labile component of normal plasma that augments the opsonization of bacteria ...
petC - Cytochrome c1 precursor - Rhodobacter capsulatus - petC gene & protein
c1 functions as an electron donor to cytochrome c. ... complex III or cytochrome b-c1 complex), which is a respiratory ... complements,/strong> the information provided at the sequence level or describes modifications for which ,strong>position- ... The main subunits of complex b-c1 are: cytochrome b, cytochrome c1 and the Rieske protein. ... c1 functions as an electron donor to cytochrome c.. Caution. The sequence reported in PubMed:3004982 was thought to originate ...
Complement component 1s - Wikipedia
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 ... involved in the complement system. C1s is part of the C1 complex.[5][6][7][8] In humans, it is encoded by the C1S gene.[9] ... complement activation, classical pathway. • immune system process. • innate immune response. • ...
Complement membrane attack complex - Wikipedia
Media related to Complement membrane attack complex at Wikimedia Commons. *Complement+Membrane+Attack+Complex at the US ... The membrane attack complex (MAC) or terminal complement complex (TCC) is a structure typically formed on the surface of ... Reid K. B. M., The complement system, in: B. D. Hames and D. M. Glover (eds.), Molecular Immunology, Oxford: IRL Press, 1988, ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ...
Activation and modulation of the first complement component, C1 :: MEDICA, MUSC Institutional Repository
Post a Comment for Activation and modulation of the first complement component, C1 ... Add tags for Activation and modulation of the first complement component, C1 ... It is speculated that the lower complement consuming activities of peptides 92 and 88 are due to the replacement of the ... Under these conditions, peptides 98 and 102 caused complement-mediated hemo~sis, while none of the other peptides tested were ...
Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation. - PubMed - NCBI
Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation.. Cai Y1, Wee SYK1, Chen J1, ... After C1 treatment, cleaved nucleolar proteins were identified by proteomic two-dimensional fluorescence difference gel ... However, C1q targets at these nucleoli to cause C1 protease activation and the cleavage of many nucleolar proteins or ... The extent of nucleolar autoantigen degradation upon C1 treatment was estimated using SLE patient autoantibodies. The isolated ...
Complement Methods and Protocols | SpringerLink
Although the complement system-a vital part of the bodys defense against bacteria-was discovered more than a century ago, its ... C1-Inhibitor: Antigenic and Functional Analysis C. Erik Hack. Pages 159-172 ... Measurement of Complement Hemolytic Activity, Generation of Complement-Depleted Sera, and Production of Hemolytic Intermediates ... Although the complement system-a vital part of the bodys defense against bacteria-was discovered more than a century ago, its ...
Activation of the first complement component (C1) by salivary bacterial agglutinins (SBA) :: MEDICA, MUSC Institutional...
Activation of the first complement component (C1) by salivary bacterial agglutinins (SBA) ... Post a Comment for Activation of the first complement component (C1) by salivary bacterial agglutinins (SBA) ... Add tags for Activation of the first complement component (C1) by salivary bacterial agglutinins (SBA) ... Activation of the first complement component (C1) by salivary bacterial agglutinins (SBA). ...
Open-Label C1 Esterase Inhibitor (C1INH-nf) for the Treatment of Acute Hereditary Angioedema (HAE) Attacks - Full Text View -...
Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... Complement C4 Serum Levels [ Time Frame: Pre-infusion to 1 hour post-infusion ]. Change in complement C4 serum levels from pre- ... Nanofiltered C1-esterase inhibitor for the acute management and prevention of hereditary angioedema attacks due to C1-inhibitor ... Open-Label C1 Esterase Inhibitor (C1INH-nf) for the Treatment of Acute Hereditary Angioedema (HAE) Attacks (CHANGE 2). The ...
Nicholson-Weller A[au] - PubMed - NCBI
Paramyosin inhibits complement C1.. Laclette JP, Shoemaker CB, Richter D, Arcos L, Pante N, Cohen C, Bing D, Nicholson-Weller A ... Increased susceptibility to endotoxin shock in complement C3- and C4-deficient mice is corrected by C1 inhibitor replacement. ... Factor J: isolation and characterization of a new polypeptide inhibitor of complement C1. ... Role of complement and complement receptor C1qR in the antibody-independent killing of group B streptococcus. ...
Complement-Related Disorders: Background, Pathophysiology, Activation
C1 inhibitor (C1-INH) inhibits C1r and C1s by binding covalently to them, causing disassembly of C1 macromolecular complex. The ... encoded search term (Complement-Related Disorders) and Complement-Related Disorders What to Read Next on Medscape. Related ... Table 3. Proteins of the Human Complement (C) System, Lectin Pathway *Table 4. Proteins of the Human Complement (C) System, C3 ... Table 1. Proteins of the Human Complement (C) System, Classical Pathway* *Table 2. Proteins of the Human Complement (C) System ...
EsteraseProteinsPathwayProteinDeficiencySerumAngioedemaAntibodyProteasesInflammationDeficienciesMarketed C1-inhibitorAntibodiesCascadePeptide Inhibitor of Complement C1ActivatesAntigenSystemPathogensComponentsRecombinantRegulatorReceptorsActivateComponent of complementInfectionComplexInhibits Complement Activation2016InflammatoryGeneInhibition of Complement ActivationSubcomponentLysisHuman complement componentInhibited complement activationMechanismBiologicalInhibitorsActivation in vitroFunctionalPeptidesHemolyticSubstratesDepositionRole of complementIndicate that complementCleavageBindActivity
Esterase40
- See Complement C1 Esterase Inhibitor [004648] . (labcorp.com)
- Absence of serum inhibitor of C1-esterase. (labcorp.com)
- Acquired C1 esterase inhibitor deficiency and angioedema: A review. (labcorp.com)
- The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and C1r subcomponent of the first component of complement by serum C1 esterase inhibitor. (labcorp.com)
- Genetically determined heterogeneity of C1 esterase inhibitor in patients with hereditary angioneurotic edema. (labcorp.com)
- What is the most important information I should know about complement C1 esterase inhibitor? (wellspan.org)
- You should not use this medicine if have ever had a life-threatening allergic reaction to complement C1 esterase inhibitor. (wellspan.org)
- Complement C1 esterase inhibitor is a man-made form of a protein in blood that helps control swelling in the body. (wellspan.org)
- Complement C1 esterase inhibitor is used in people with hereditary angioedema. (wellspan.org)
- Complement C1 esterase inhibitor may also be used for purposes not listed in this medication guide. (wellspan.org)
- What should I discuss with my healthcare provider before using complement C1 esterase inhibitor? (wellspan.org)
- Complement C1 esterase inhibitor is made from donated human plasma and may contain viruses or other infectious agents. (wellspan.org)
- How should I use complement C1 esterase inhibitor? (wellspan.org)
- Complement C1 esterase inhibitor contains no preservative. (wellspan.org)
- What should I avoid after using complement C1 esterase inhibitor? (wellspan.org)
- What are the possible side effects of complement C1 esterase inhibitor? (wellspan.org)
- The Effectiveness and Value of Lanadelumab and C1 Esterase Inhibitors for Prophylaxis of Hereditary Angioedema Attacks. (harvard.edu)
- Recently we have studied the ability of an enzymatically modified form of LDL (E-LDL), obtained by treatment with trypsin and then cholesterol esterase, to activate C1, the complex that triggers activation of the classical pathway of complement. (europa.eu)
- In addition to trypsin, plasmin, thrombin, tryptase and matrix metalloprotease-2 each yielded E-LDL particles with high C1-activating efficiency, and the C1 activation extent was strictly dependent on cholesterol esterase treatment in all cases. (europa.eu)
- When incorporated into vesicles, the lipid fraction of E-LDL, but not of native LDL, triggered C1 activation, and activation correlated with the amount of free cholesterol released by cholesterol esterase. (europa.eu)
- plasma is possible) to detect and/or measure levels of alpha-2-globulin, an esterase inhibitor of the C1 protein found in the classic pathway of the complement components proteins. (optometricmanagement.com)
- Complement component 1s ( EC 3.4.21.42 , C1 esterase , activated complement C1s , complement C overbar 1r , C1s ) is a protein involved in the complement system . (wikipedia.org)
- This then acts similarly to C1 esterase and cleaves C2 and C4 to form C2aC4b, which is the C3 esterase that cleaves C3 to form C3b. (medscape.com)
- C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood. (medlineplus.gov)
- Your health care provider will also measure the functional activity level of your C1 esterase inhibitor. (medlineplus.gov)
- Objectives: To determine serum concentrations of proinflammatory (C reactive protein, complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite cyclists before and during a three week cycle tour. (edu.au)
- abstract = "Objectives: To determine serum concentrations of proinflammatory (C reactive protein, complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite cyclists before and during a three week cycle tour.Methods: Seventeen professional cyclists participating in the Vuelta a Espańa volunteered for the study. (edu.au)
- C1-esterase inhibitor (C1-inh) is a serine protease inhibitor (SERPIN) that acts by forming a complex with active enzymes to trap and inactivate them. (primaryimmune.org)
- This document contains side effect information about C1 esterase inhibitor (human). (drugs.com)
- Along with its needed effects, C1 esterase inhibitor (human) may cause some unwanted effects. (drugs.com)
- Some side effects of C1 esterase inhibitor (human) may occur that usually do not need medical attention. (drugs.com)
- Cinryze (complement C1 esterase inhibitor). (drugs.com)
- 2. Gandhi PK, Gentry WM, Bottorff MB "Thrombotic events associated with C1 esterase inhibitor products in patients with hereditary angioedema: investigation from the United States Food and Drug Administration adverse event reporting system database. (drugs.com)
- HAE is caused by a shortage of a protein called C1 esterase inhibitor, that is present in your blood and helps control inflammation (swelling) and parts of the immune system . (rxlist.com)
- A shortage of C1 esterase inhibitor can lead to repeated attacks of swelling, pain in the abdomen, difficulty breathing and other symptoms. (rxlist.com)
- RUCONEST is a recombinant analogue of human complement component 1 esterase inhibitor for intravenous injection. (rxlist.com)
- One IU of rhC1INH activity is defined as the equivalent of C1 esterase inhibiting activity present in 1 mL of pooled normal plasma. (rxlist.com)
- The primary and secondary structures of the molecule and target protease selectivity are consistent with those of plasma-derived C1 esterase inhibitor. (rxlist.com)
- One such factor is complement C1 esterase inhibitor or C1INH. (medpagetoday.com)
- Tradtrantip L, Asavapanumas N, Phuan PW, Verkman AS (2014) Potential therapeutic benefit of C1-esterase inhibitor in neuromyelitis optica evaluated in vitro and in an experimental rat model. (omicsonline.org)
Proteins48
- Deposition of complement proteins on the bacteria was monitored by Western blotting. (asm.org)
- The complement system is a series of proteins that act in a defined sequence (Fig. 1 ) to promote immune clearance by opsonizing or killing microorganisms and augmenting the inflammatory response. (asm.org)
- The nonactivated complement proteins are indicated above and to the right of the wide arrows, and the arrows pointing from them show the product that is released during activation. (asm.org)
- To elucidate the molecular basis for complement resistance by BrkA, in this study we have attempted to determine which step in the complement cascade is affected by BrkA by monitoring the deposition of complement proteins on the surface of strains either expressing or not expressing BrkA. (asm.org)
- The complement system is made up of a large number of distinct plasma proteins that react with one another to opsonize pathogens and induce a series of inflammatory responses that help to fight infection. (nih.gov)
- A number of complement proteins are proteases that are themselves activated by proteolytic cleavage. (nih.gov)
- In this way, the activation of a small number of complement proteins at the start of the pathway is hugely amplified by each successive enzymatic reaction, resulting in the rapid generation of a disproportionately large complement response. (nih.gov)
- First, it generates large numbers of activated complement proteins that bind covalently to pathogens, opsonizing them for engulfment by phagocytes bearing receptors for complement. (nih.gov)
- Second, the small fragments of some complement proteins act as chemoattractants to recruit more phagocytes to the site of complement activation, and also to activate these phagocytes. (nih.gov)
- 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)
- Broad Susceptibility of Nucleolar Proteins and Autoantigens to Complement C1 Protease Degradation. (cdc.gov)
- Considering a recent finding that, in dead cells, nucleoli were targeted by C1q and two nucleolar autoantigens were degraded by C1r/C1s proteases, we considered that C1 could help protect against antinuclear autoimmunity by broadly degrading nucleolar proteins or autoantigens. (cdc.gov)
- After C1 treatment, cleaved nucleolar proteins were identified by proteomic two-dimensional fluorescence difference gel electrophoresis and mass spectrometry, and further verified by Western blotting using specific Abs. (cdc.gov)
- 20 nucleolar proteins were identified as C1 cleavable. (cdc.gov)
- However, C1q targets at these nucleoli to cause C1 protease activation and the cleavage of many nucleolar proteins or autoantigens. (cdc.gov)
- The complement system as understood today is a multimolecular system composed of more than 32 proteins and consisting of serum proteins, serosal proteins, and cell membrane receptors that bind to complement fragments. (medscape.com)
- The complement system consists of 7 serum and 9 membrane regulatory proteins, 1 serosal regulatory protein, and 8 cell membrane receptors that bind complement fragments. (medscape.com)
- The complement system is an organized proteolytic cascade of dozens of proteins that functions in the recognition, opsonization, and lysis of pathogenic and altered-host cells. (ecu.edu)
- 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)
- When the complement system is turned on during inflammation, levels of complement proteins may go down. (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 inflammatory mediators, cytokines and complement proteins are believed to regulate the sequential events during the development. (elsevier.com)
- In the present study, expression of complement proteins (C1 inhibitor, factor H, factor B, C4) by cultured endothelial cells obtained from human brain microvessels has been characterized. (elsevier.com)
- Serine proteases, plasmin and miniplasmin induce the expression of C4, decrease the level of ELISA detectable C1 inhibitor, and do not affect the production of factors H and B. These data indicate that complement proteins are expressed locally by the brain microvessels, and may modulate the inflammatory responses of brain tissue. (elsevier.com)
- Complement is the term used to describe a group of serum proteins that are critically important in our defense against infection. (primaryimmune.org)
- The complement system consists of more than 30 proteins, present in blood and tissues, as well as other proteins anchored on the surfaces of cells. (primaryimmune.org)
- Complement proteins in the circulation are not activated until triggered by an encounter with a bacterial cell, a virus, an immune complex, damaged tissue or other substance not usually present in the body. (primaryimmune.org)
- Several studies indicate that complement proteins exert functions that are either more complex than previously thought, or go well beyond the innate immune character of the system. (springer.com)
- The complement consists of at least 35 or more plasma proteins and cell surface receptors/regulators, which can be activated by three pathways: classical (CP), lectin (LP), and alternative (AP). (frontiersin.org)
- Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. (wikipedia.org)
- The diagnostic tests used to diagnose a complement deficiency include: CH50 measurement Immunochemical methods/test C3 deficiency screening Mannose-binding lectin (lab study) Plasma levels/regulatory proteins (lab study) Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema. (wikipedia.org)
- Disorders of the proteins that act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections. (wikipedia.org)
- C1-inh is the largest member among the serpin class of proteins. (bionity.com)
- During inflammation, vascular permeability is increased by various proteolytic events, such as the generation of bradykinin, that augment local tissue responses by enabling tissue penetration of serum proteins, including complement and acute-phase proteins. (sciencemag.org)
- Of the proteins identified in normal skin, about half were cleaved, and phorbol ester-induced inflammation increased the proportion of cleaved proteins, including chemokines and complement proteins, that were processed at previously uncharacterized sites. (sciencemag.org)
- Complement comprises 25 to 30 discrete proteins, labeled numerically as C1 to C9, and by letters, i.e. (thefreedictionary.com)
- The complement system consists of a number of small proteins found in the blood, normally circulating as inactive zymogens . (wikidoc.org)
- Over 20 proteins and protein fragments make up the complement system, including serum proteins, serosal proteins, and cell membrane receptors. (wikidoc.org)
- Kirjavainen V, Jarva H, Biedzka Sarek M, Blom A, Skurnik M, Meri S. Yersinia enterocolitica serum resistance proteins YadA and ail bind the complement regulator C4b-binding protein. (labome.org)
- At the cell surface is thought to act as an endothelial receptor for plasma proteins of the complement and kallikrein-kinin cascades. (uniprot.org)
- These proteins are part of your complement system, an important part of your immune system that helps kill disease-causing bacteria and viruses. (rochester.edu)
Pathway34
- Objective- The classical complement pathway has been assigned both protective and pathological effects in cardiovascular disease (CVD), but human data are lacking. (ahajournals.org)
- These studies show that BrkA inhibits the classical pathway of complement activation and prevents accumulation of deposited C4. (asm.org)
- Antigen-antibody complexes on the surface of a microorganism can activate the classical pathway of complement, a part of the acquired immune system (Fig. 1 ), by providing a binding site for C1. (asm.org)
- Mannose binding protein activates the classical pathway by binding to mannose residues on microbial surfaces and activating C4 in a manner similar to that for C1. (asm.org)
- Both the classical and alternative pathways of complement activation are represented, although details are given only for the classical pathway. (asm.org)
- Interestingly, B. pertussis does not activate the alternative pathway of complement ( 7 ). (asm.org)
- We have recently developed a rat model of acute intravascular hemolysis showing that the classical complement pathway mediates antibody-dependent hemolysis. (elsevier.com)
- The objective of this study was to evaluate the role of the classical pathway inhibitor peptide inhibitor of complement C1 (PIC1) in this animal model. (elsevier.com)
- complement protein in the pathway, C1 , must interact with a bound immunoglobulin. (britannica.com)
- This in turn cleaves and activates the next zymogen in the complement pathway. (nih.gov)
- The classical pathway can be initiated by the binding of C1q, the first protein in the complement cascade, directly to the pathogen surface. (nih.gov)
- Finally, the alternative pathway can be initiated when a spontaneously activated complement component binds to the surface of a pathogen. (nih.gov)
- Conflicting reports exist in the literature concerning the primary mechanism for heparin's inhibition or classical complement pathway activity . (musc.edu)
- Depending on the nature of complement activators, the classic pathway, the alternative pathway, or the more recently discovered lectin pathway is activated predominantly to produce C3 convertase. (medscape.com)
- Binding of factor H to C3b increases its inactivation by factor I. Properdin stabilizes it, preventing its inactivation by factors H and I. The alternate pathway does not result in a truly nonspecific activation of complement because it requires specific types of compounds for activation. (medscape.com)
- Classical Complement Pathway Component C1q: Purification of Human C1q, Isolation of C1q Collagen-Like and Globular Head Fragments and Production of Recombinant C1q-Derivatives. (springer.com)
- Total complement activity (CH50 or CH100) may be ordered to look at the integrity of the entire classical complement pathway. (labcorp.com)
- Hemolytic assays utilizing sera depleted of individual complement factors as well as adding back purified factors demonstrated that HAstV CP suppresses classical pathway activation at the first component, C1. (realtalifesciences.com)
- Although diverse mechanisms of inhibition of complement activation have been described for many enveloped animal viruses, this is the first report of a nonenveloped icosahedral virus CP inhibiting classical pathway activation at C1. (realtalifesciences.com)
- causes the activation of the classical complement (C) pathway in normal human serum, but not in guinea pig serum. (elsevier.com)
- C1r is a modular serine protease which is the autoactivating component of the C1 complex of the classical pathway of the complement system. (elsevier.com)
- The Alternative Pathway (AP) is initiated by fragments of the complement component C3. (primaryimmune.org)
- This time-limitation is another control mechanism for the complement pathway. (primaryimmune.org)
- The Terminal Pathway (TP) is the final set of steps in the complement activation process that forms a membrane lesion or hole (membrane attack complex or MAC) that kills susceptible bacteria or other cells that activate complement on their surfaces. (primaryimmune.org)
- In the classical pathway (CP), this is mainly mediated by binding of the C1 complex, consisting of the PRM C1q and the proteases C1r and C1s, to Ig patches on the pathogen. (jimmunol.org)
- Recent investigations have shed light on the role of lectin complement pathway in septic shock. (hindawi.com)
- Different initiators activate each pathway but all converge to complement protein C3 and are followed by a common cascade (C5-9), resulting in the deposition of a membrane-attack-complex on targets and the release of chemoattractants (C3a and C5a) for inflammatory cells. (hindawi.com)
- The classical complement pathway plays a major role in innate immunity against infection. (novusbio.com)
- This pathway is triggered by C1, a multimolecular complex composed of the recognition protein C1q and two serine proteases, C1r and C1s. (novusbio.com)
- the sequence of reactions, each being the catalyst for the next, that leads to the terminal complement pathway and cell lysis. (thefreedictionary.com)
- Three biochemical pathways activate the complement system: the classical complement pathway , the alternative complement pathway , and the mannose-binding lectin pathway . (wikidoc.org)
- The classical complement pathway typically requires antibodies for activation (specific immune response), while the alternative and mannose-binding lectin pathways can be activated by C3 hydrolysis or antigens without the presence of antibodies (non-specific immune response). (wikidoc.org)
- The classical pathway is triggered by activation of the C1-complex (which consists of one molecule C1q and two molecules C1r and C1s), either by C1q's binding to antibodies from classes M and G, complexed with antigens , or by its binding C1q to the surface of the pathogen. (wikidoc.org)
- There is wide variation of infections associated with complement deficiency depending on which complement protein and activation pathway is affected(Skattum et al 2011). (pediatriconcall.com)
Protein30
- Complement C1 Inhibitor Protein" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (harvard.edu)
- This graph shows the total number of publications written about "Complement C1 Inhibitor Protein" by people in Harvard Catalyst Profiles by year, and whether "Complement C1 Inhibitor Protein" was a major or minor topic of these publication. (harvard.edu)
- Below are the most recent publications written about "Complement C1 Inhibitor Protein" by people in Profiles. (harvard.edu)
- Bordetella pertussis produces a 73-kDa protein, BrkA (Bordetella resistance to killing), which inhibits the bactericidal activity of complement. (asm.org)
- For example, the mannose binding protein is a pattern recognition molecule with structural similarity to C1 that bridges the classical and alternative pathways ( 18 ). (asm.org)
- CFH / Complement Factor H is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. (lsbio.com)
- This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. (lsbio.com)
- The 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)
- The effect of 15 U C1-INH (intravenously) was evaluated in terms of general and focal neurologic deficits, ischemic volume, neutral red staining (to identify the brain areas subject to ischemic damage), and glial fibrillary acidic protein immunoreactivity (to show astrocytic response). (gwu.edu)
- It controls a protein called C1, which is part of the complement system. (medlineplus.gov)
- This regulatory protein is a fluid phase serine protease that controls proenzyme activation and enzyme activity of not only the complement system but also the contact system. (uu.nl)
- Complement C3 is a blood test that measures the activity of a certain protein. (medlineplus.gov)
- This protein is part of the complement system. (medlineplus.gov)
- Here we demonstrate that HAstV type 1 (HAstV-1) virions, specifically the viral coat protein (CP), suppress the complement system, a fundamental component of the innate immune response in vertebrates. (realtalifesciences.com)
- This fragment contains the C-terminal serine protease (SP) domain and the preceding two complement control protein (CCP) modules. (elsevier.com)
- Patients with complement deficiencies encounter clinical problems that depend on the role of the specific complement protein in normal function. (primaryimmune.org)
- Complement research is now faced with the challenge of trying to integrate isolated biochemical pathways into complex gene and protein regulatory circuits. (springer.com)
- This volume represents a collection of topics on the "novel" functions of complement, patho-physiology, protein structures, design of complement inhibitors, and complement assays discussed during the conference. (springer.com)
- The hublike organization of complement and its cell surface-directed action ( Fig. 1 ), involving some 50 constituents such as pattern-recognition molecules (PRM), protein components, proteases, regulators, and cell surface receptors, is essential for adjusting the complement response to different triggers ( Fig. 2A ). (jimmunol.org)
- The occurrence of soluble immune complexes (IC) in the cerebrospinal fluid (CSF) of 14 multiple sclerosis (MS) patients, four acute polyradiculoneuritis patients, 30 patients with other neurological diseases (OND) and 30 patients with disc prolapse (DP) was examined by a solid phase C1q-protein A binding assay (C1q-PABA) and a complement consumption test. (biomedsearch.com)
- C1-inh is an acute phase protein, it circulates in blood at levels of around 0.25 g/l, which levels rise ~2-fold during inflammation. (bionity.com)
- In 85% of the cases, the levels of C1-inh are low, while in 15% the protein circulates in normal amounts but it is dysfunctional. (bionity.com)
- A complement protein attacking an invader. (wikidoc.org)
- Complement receptor type 1 ( CR1 ) also known as C3b/C4b receptor or CD35 (cluster of differentiation 35) is a protein that in humans is encoded by the CR1 gene . (wikidoc.org)
- The protein mediates cellular binding to particles and immune complexes that have activated complement. (wikidoc.org)
- In 5'-3' order the genes in this region are: membrane cofactor protein - CR1 - complement receptor type 2 - decay-accelerating factor - C4-binding protein. (wikidoc.org)
- Meri T, Blom A, Hartmann A, Lenk D, Meri S, Zipfel P. The hyphal and yeast forms of Candida albicans bind the complement regulator C4b-binding protein. (labome.org)
- The MicroVue C1 Inhibitor Plus Enzyme Immunoassay (EIA) measures functional levels of C1 Inhibitor protein present in human plasma or serum. (quidel.com)
- Here we present evidence indicating that antibodies able to enhance infectivity of authentic Ebola Zaire virus are produced in humans and propose a novel mechanism for this antibody-dependent enhancement (ADE) in which the complement protein C1q mediates the enhancement. (asm.org)
Deficiency20
- C1-inhibitor deficiency and angioedema. (labcorp.com)
- Although the causes of these Abs remain broad and ill-defined, a genetic deficiency in C1 complex (C1qC1r 2 C1s 2 ) or C4 is able to induce these Abs. (cdc.gov)
- This may represent one important surveillance mechanism against antinuclear autoimmunity because C1 genetic deficiency causes anti-nuclear autoantibodies and SLE disease. (cdc.gov)
- Diagnosis, course, and management of angioedema in patients with acquired C1-inhibitor deficiency. (medlineplus.gov)
- 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)
- The following symptoms (signs) are consistent with complement deficiency in general: Recurring infection Auto-immune disorders Glomerulonephritis Joint problems (manifestation) Lung function (MBL variant alleles) Angioedema Dermatomyositis Vasculitis Anaphylactoid purpura Play media Vaccinations for encapsulated organisms (e.g. (wikipedia.org)
- The cause of complement deficiency is genetics (though cases of an acquired nature do exist post infection). (wikipedia.org)
- The majority of complement deficiencies are inherited as autosomal recessive conditions, while properdin deficiency occurs through X-linked inheritance. (wikipedia.org)
- C1-inhibitor deficiency or hereditary angioedema will have low C4 with normal C1 levels. (wikipedia.org)
- The mechanism of complement deficiency consists of: C2: In regard to C2 deficiency, about 5 different mutations in the C2 gene are responsible. (wikipedia.org)
- In terms of management for complement deficiency, immunosuppressive therapy should be used depending on the disease presented. (wikipedia.org)
- A C1-INH concentrate can be used for angio-oedema (C1-INH deficiency). (wikipedia.org)
- Epsilon-aminocaproic acid could be used to treat hereditary C1-INH deficiency, though the possible side effect of intravascular thrombosis should be weighed. (wikipedia.org)
- Deficiency of C1-inh permits plasma kallikrein activation, which leads to the production of the vasoactive peptide bradykinin. (bionity.com)
- Hereditary angioedema (HAE) with C1 inhibitor deficiency manifests as recurrent episodes of edema involving the skin, upper respiratory tract and gastrointestinal tract. (scielo.br)
- C1 INH deficiency with prevalence of approximately 1 in 50 000 presents as hereditary angioedema. (pediatriconcall.com)
- You can inherit a deficiency in your complement C4, but it is much more common to acquire a deficiency. (rochester.edu)
- If only your C4 complement level is low, and all other complement components are normal, it is usually because of an inherited component deficiency. (rochester.edu)
- A deficiency in complement C4 levels has been linked to different forms of kidney disease and chronic hepatitis. (rochester.edu)
Serum16
- To improve upon the solubility of the lead derivative, a pegylated, water soluble variant was developed, structurally characterized and demonstrated to inhibit complement activation in mouse plasma, as well as rat, non-human primate and human serum in vitro. (nih.gov)
- Whereas treatment of E-LDL particles with human serum albumin reduced their free fatty acid content, both cholesterol and free fatty acids were decreased by methyl-beta cyclodextrin, both treatments resulting in a dose-dependent inhibition of the C1-activating ability of the particles. (europa.eu)
- Although often overlooked as a defense of the respiratory tract, complement levels in this location are normally 10 to 20% of that found in serum and increase during inflammation ( 21 ). (asm.org)
- C3 complement (beta-1c-globulin) - serum. (medlineplus.gov)
- HAstV-1 CP bound the A chain of C1q and inhibited serum complement activation, resulting in decreased C4b, iC3b, and terminal C5b-9 formation. (realtalifesciences.com)
- To investigate why C was not activated in guinea pig serum, we partially purified guinea pig C1 in the presence of the protease inhibitor p-nitrophenyl, p'-guanidinobenzoate (NPGB). (elsevier.com)
- The PS decreased the action of the C1 inhibitor for C1 in diluted guinea pig serum, and it also inhibited the activity of highly purified guinea pig C1 inhibitor for C1. (elsevier.com)
- The activity of the human C1 inhibitor, either in diluted serum or highly purified, was not decreased by the PS. (elsevier.com)
- These results show that the PS does not activate guinea pig C1 in serum because its action is blocked by the C1 inhibitor. (elsevier.com)
- Schultz, DR & Arnold, PI 1981, ' Activation of first component of complement (C1) in guinea pig serum by a polysaccharide is prevented by C1 inhibitor ', Journal of Immunology , vol. 126, no. 4, pp. 1558-1561. (elsevier.com)
- Arnold, P. I. / Activation of first component of complement (C1) in guinea pig serum by a polysaccharide is prevented by C1 inhibitor . (elsevier.com)
- In the first, also referred to as the test system, antigen is mixed usually with serial dilutions of a test serum in the presence of complement. (thefreedictionary.com)
- If the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. (thefreedictionary.com)
- The MicroVue CH50 Eq EIA is designed to measure total hemolytic complement (CH50) in human serum samples. (quidel.com)
- Plasma or serum from convalescing patients enhanced the infection of primate kidney cells by the Zaire virus, and this enhancement was mediated by antibodies to the viral glycoprotein and by complement component C1q. (asm.org)
- 293 and Vero E6 cells were grown in Dulbecco's minimal Eagle's medium complemented with 10% fetal bovine serum, l -glutamine, and antibiotics. (asm.org)
Angioedema8
- A multicentre study to investigate pharmacokinetics, clinical efficacy and safety of nanofiltered Cetor® (called C1-esteraseremmer-N during the development phase) for the treatment of hereditary angioedema (HAE) will be performed. (clinicaltrials.gov)
- This study KB2003.01 consists of three parts, part A pharmacokinetics (phase II), part B treatment of attacks of angioedema (phase III) and part C prophylactic use of C1 inhibitor (phase III). (clinicaltrials.gov)
- In part A, the pharmacokinetics of C1-esteraseremmer-N in patients with hereditary angioedema will be compared with the current registered product, Cetor®, in a randomised, blinded cross-over design. (clinicaltrials.gov)
- Both forms of angioedema are caused by low levels of C1-INH. (medlineplus.gov)
- Low levels of C1-INH may cause certain types of angioedema. (medlineplus.gov)
- There are two types of angioedema that result from decreased levels of C1-INH. (medlineplus.gov)
- Despite uncontrolled auto-activation, it is important to note that levels of key complement components are low during an acute attack, because they are being consumed - indeed, low levels of C4 are a key diagnostic test for hereditary angioedema. (bionity.com)
- O angioedema hereditário (AEH) com deficiência de inibidor de C1 manifesta-se por episódios recorrentes de edema envolvendo pele, trato respiratório superior e gastrointestinal. (scielo.br)
Antibody8
- Complement Factor H antibody LS-C707907 is an HRP-conjugated mouse monoclonal antibody to rat Complement Factor H (CFH). (lsbio.com)
- Specifically, C1 interacts with the tail of the Y portion of the bound antibody molecule-i.e., the nonspecific part of the antibody that does not bind antigen. (britannica.com)
- This activity was said to ' complement ' the antibacterial activity of antibody , hence the name. (nih.gov)
- Although first discovered as an effector arm of the antibody response, complement can also be activated early in infection in the absence of antibodies. (nih.gov)
- There are currently no images for Complement Component C1r Antibody (AF1807). (novusbio.com)
- There are currently no images for Complement Factor H Antibody (NBP2-23542AF700). (novusbio.com)
- If antibody was not present in stage 1, then the free complement lyses the sensitized sheep red blood cells. (thefreedictionary.com)
- Complement and anticomplement antibody. (asm.org)
Proteases2
- Using surface plasmon resonance, ErpB and ErpQ were found to bind C1 complex proteases C1r and C1s with high affinity. (ecu.edu)
- Mechanistic studies on ErpB and ErpQ also support further understanding of molecular interactions between complement proteases and their substrates, which provides alternative means for the development of specific complement therapeutics toward complement-mediated diseases. (ecu.edu)
Inflammation7
- We determined the associations of the pattern recognition factor C1q and the regulator C1-INH with incident CVD, carotid intima-media thickness, endothelial dysfunction, and low-grade inflammation. (ahajournals.org)
- 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 HD, Gupta G. Mediators of inflammation: complement. (medlineplus.gov)
- The specific aim of this study is to evaluate the effect of recombinant human C1-inhibitor (rhC1INH), as a kidney recipient intra- and post operative treatment strategy to decrease systemic inflammation and decrease the incidence of DGF from donation after cardiac death donors (DCD). (clinicaltrials.gov)
- Since complement is a major contributor to the initiation and amplification of inflammation, the observed CP-mediated inhibition of complement activity may contribute to the lack of inflammation associated with astrovirus-induced gastroenteritis. (realtalifesciences.com)
- Does complement icreases after inflammation? (brainscape.com)
- Complement has long been regarded as a pivotal effector arm of the innate immune response, eliciting important immunoregulatory functions in the context of inflammation and also serving as a vital link between the innate and adaptive immune response. (springer.com)
Deficiencies12
- 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)
- C3 and C4 levels are the most frequently ordered, but others, such as C1 inhibitor, may be ordered when other deficiencies are suspected. (labcorp.com)
- There are deficiencies of each of the individual components of complement. (primaryimmune.org)
- Neisseria meningitidis and Streptococcus pneumoniae) is crucial for preventing infections in complement deficiencies. (wikipedia.org)
- medical citation needed] Among the possible complications are the following: Deficiencies of the terminal complement components increases susceptibility to infections by Neisseria. (wikipedia.org)
- What are complement deficiencies? (wikipedia.org)
- Some complement deficiencies caries risk of infection, other mainly associated with autoimmune diseases. (pediatriconcall.com)
- Some genetic and molecular defects has been identified to be associated complement deficiencies. (pediatriconcall.com)
- Complement deficiencies and associated genetic disorders and clinical manifestations is summarized in Table 1. (pediatriconcall.com)
- Table 1 - Complement deficiencies and clinical associations. (pediatriconcall.com)
- The measurement of CH50 allows detection of deficiencies of one or more complement. (quidel.com)
Marketed C1-inhibitor1
- The changes in the manufacturing process of C1-esteraseremmer-N, compared to Cetor® (the currently marketed C1-inhibitor product), nanofiltration and omission of hepatitis B immunoglobulin, most likely will not affect tolerability. (clinicaltrials.gov)
Antibodies7
- Complement was discovered many years ago as a heat-labile component of normal plasma that augments the opsonization of bacteria by antibodies and allows antibodies to kill some bacteria. (nih.gov)
- Researchers in the Sharp lab solved the structures of IgM antibodies with multiple components of the immune complement system, revealing insights into immune system activation. (lumc.nl)
- IgM antibodies protect us against infections and mediate clearance of cellular debris by activating the immune complement system upon binding to cell-surface antigens. (lumc.nl)
- were able to image activation of the immune system complement cascade by IgM antibodies. (lumc.nl)
- In the early 20th century, this controversy was resolved when it was understood that complement can act in combination with specific antibodies, or on its own in a non-specific way. (wikidoc.org)
- Monitoring patients with the C1q assay, which detects antibodies that fix complement, offers a minimally invasive means of identifying patients at risk for transplant glomerulopathy and graft loss. (labome.org)
- 05). The results of our study suggest that anti-beta2GPI antibodies may play a role in GMT formation, and this process might involve complement activation. (labome.org)
Cascade14
- In this study we characterized the step in the complement cascade where BrkA acts, using three strains: a wild-type strain, a strain containing an insertional disruption of brkA , and a strain containing two copies of the brkA locus. (asm.org)
- The complement cascade. (asm.org)
- The complement system activates through a triggered-enzyme cascade. (nih.gov)
- In such a cascade, an active complement enzyme generated by cleavage of its zymogen precursor then cleaves its substrate, another complement zymogen, to its active enzymatic form. (nih.gov)
- Schematic overview of the complement cascade. (nih.gov)
- In the early phases of an infection, the complement cascade can be activated on the surface of a pathogen through any one, or more, of the three pathways shown in Fig. 2.8 . (nih.gov)
- The complement system functions as an interactive sequence, with one reaction leading to another in the form of a cascade. (medscape.com)
- C1s then enzymatically cleaves C4 to form C4b (cyan) and initiate the complement cascade. (lumc.nl)
- The complement cascade is a series of reactions that take place in the blood. (medlineplus.gov)
- The Classical Complement Cascade Mediates CNS Synapse Elimination. (freepatentsonline.com)
- The activation of the complement cascade can cause damage to cells, therefore the inhibition of the complement cascade can work as a medicine in certain conditions [1] . (bionity.com)
- Basic and clinical studies suggest that activation of complements in the common cascade, for example, complement component 3 (C3) and C5, is involved in the development of septic shock. (hindawi.com)
- The complement system is a biochemical cascade which helps clear pathogens from an organism. (wikidoc.org)
- It has been shown that CR1 can act as a negative regulator of the complement cascade, mediate immune adherence and phagocytosis and inhibit both the classic and alternative pathways. (wikidoc.org)
Peptide Inhibitor of Complement C11
- Our laboratory has identified a peptide that specifically inhibits the classical and lectin pathways of complement and is referred to as Peptide Inhibitor of Complement C1 (PIC1). (nih.gov)
Activates3
- The results indicate a surface trigger that activates complement and contributes to differentiating between silent removal of targeted host cells versus raising inflammatory responses to invading microbes. (lumc.nl)
- Following the C1q recognition, C1r is autoactivated, and in turn activates C1s, which cleaves C4 and C2, the C1 substrates (1). (novusbio.com)
- Structures of C1-IgG1 provide insights into how danger pattern recognition activates complement. (pdbj.org)
Antigen1
- Ehrlich believed that each antigen-specific amboceptor had its own specific complement, while Bordet believed that there is only one type of complement. (wikidoc.org)
System37
- The complement system has been increasingly recognized to play a pivotal role in a variety of inflammatory and autoimmune diseases. (nih.gov)
- Consequently, therapeutic modulators of the classical, lectin and alternative pathways of the complement system are currently in pre-clinical and clinical development. (nih.gov)
- Complement, a complex system of enzymes and regulators endowed with multiple biological activities (opsonisation, chemoattraction of leukocytes, cell lysis, cell activation), is a major component of innate defence against pathogens. (europa.eu)
- Indeed, it now seems clear that complement first evolved as part of the innate immune system , where it still plays an important role. (nih.gov)
- In the case of the complement system, the precursor zymogens are widely distributed throughout body fluids and tissues without adverse effect. (nih.gov)
- There are three ways in which the complement system protects against infection. (nih.gov)
- All three pathways of the complement system ( classical , lectin and alternative pathways) initiate the formation of MAC. (wikipedia.org)
- Reid K. B. M., The complement system , in: B. D. Hames and D. M. Glover (eds. (wikipedia.org)
- A series of Cl consumption , C2 destruction and C3 conversion tests were used to determine the most effective sequence for complement activation and to gain information about the functional nature of the interactions between the peptides and the human complement system . (musc.edu)
- Although the complement system-a vital part of the body's defense against bacteria-was discovered more than a century ago, its study has undergone a renaissance with the identification of its regulatory molecules and the realization that these molecules can be used therapeutically. (springer.com)
- In Complement Methods and Protocols, B. Paul Morgan and a team of expert laboratorians present a comprehensive set of readily reproducible methods to study this critical system. (springer.com)
- These reactions could have biological importance on inflamed mucosal surfaces by providing an effective means for complement activation and deposition on SBA coated microorganisms which might otherwise be resistant to the complement system . (musc.edu)
- Atkinson JP, Du Clos TW, Mold C, Kulkarni H, Hourcade D, Wu X. The human complement system: basic concepts and clinical relevance. (medlineplus.gov)
- We demonstrate that recombinant as well as endogenous Vag8 enhanced contact system activity by binding C1-INH and attenuating its inhibitory function. (uu.nl)
- 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)
- Complement system in disease. (medlineplus.gov)
- The primary functions of the complement system are to protect from infection, to remove particulate substances, (like damaged or dying cells, microbes or immune complexes) and to help modulate adaptive immune responses. (primaryimmune.org)
- As part of the innate immune system, complement acts immediately to start the process of removal and resolution of the problem. (primaryimmune.org)
- Complement works with the inflammatory cells of the innate immune system and those of adaptive or acquired immunity. (primaryimmune.org)
- A unique feature of the AP is the presence of the only positive regulator in the complement system, Properdin. (primaryimmune.org)
- Complement C[bdi2] inhibitor (inactivator) immunological test system. (fda.gov)
- They have led complementologists to revisit established biological systems, such as the complement system, from a global and integrative perspective. (springer.com)
- In recent years, the complement system has been associated with a growing number of immunological and inflammatory conditions that include degenerative diseases, cancer, and transplant rejection. (jimmunol.org)
- Given the classic perception of complement as defense system against microbial intruders, it may appear surprising that this ancient pillar of innate immunity was identified as a contributor in various inflammatory pathologies. (jimmunol.org)
- Because of redundancies in the immune system, many complement disorders are never diagnosed. (wikipedia.org)
- Also, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. (bionity.com)
- The complement system plays a major role in the host's defence against infections and in immune complex diseases. (indigo.ca)
- This fully revised edition of Complement in Health and Disease provides an up-to-date account of how the system works and its effects on the host. (indigo.ca)
- While complement is an important defense system against bacterial infection, earlier clinical observations suggest that activation of complement factors is associated with detrimental effects in septic shock, such as multiorgan damages and poor outcome [ 5 - 8 ]. (hindawi.com)
- There are three pathways in the complement system: classical, alternative, and lectin. (hindawi.com)
- The complement system is known to be activated by the immunoglobulins IgM and IgG. (thefreedictionary.com)
- The term "complement" was introduced by Paul Ehrlich in the late 1890s, as part of his larger theory of the immune system. (wikidoc.org)
- Ehrlich therefore named this heat-labile component "complement", because it is something in the blood which "complements" the cells of the immune system. (wikidoc.org)
- In primates, CR1 serves as the main system for processing and clearance of complement opsonized immune complexes . (wikidoc.org)
- By measuring complement C4 levels, especially in how they compare with other parts of the complement system, your healthcare provider can diagnose and monitor treatment of certain diseases. (rochester.edu)
- This test measures all nine components of the complement system, from C1 to C9. (rochester.edu)
- When your complement system is activated in response to ongoing disease such as lupus, levels usually go down. (rochester.edu)
Pathogens9
- Complement is also part of the innate immune defenses and provides a defense against pathogens that have not previously infected the host by recognizing repeating structures such as lipopolysaccharide (LPS) found on the surface of bacteria. (asm.org)
- Complement resistance is common among respiratory pathogens. (asm.org)
- Bloodborne pathogens like the etiologic agent of Lyme disease, Borrelia burgdorferi, encounter complement during their bloodmeal and in their dissemination through the body. (ecu.edu)
- Therefore, to avoid complement mediated destruction, these pathogens have developed mechanisms that aid in complement evasion and defense. (ecu.edu)
- The discovery of a novel mechanism of complement inhibition by a medically-relevant human pathogen expands our knowledge of host pathogens interactions and contributes to previously unknown pathophysiological immune evasion by B. burgdorferi. (ecu.edu)
- Inhibition of complement activation was also demonstrated for HAstV serotypes 2 to 4, suggesting that this phenomenon is a general feature of these human pathogens. (realtalifesciences.com)
- Yet it has become evident that complement not only acts as a sensor of pathogens but also recognizes diseased and damaged host cells, and it closely collaborates with other immune and defense systems to eliminate potential danger ( 1 , 2 ). (jimmunol.org)
- The past decade revealed a new perception of complement that reaches beyond the elimination of pathogens and includes key functions in immune surveillance, homeostasis, and mediation of inflammatory responses ( 1 , 2 ). (jimmunol.org)
- Kupffer cells and other macrophage cell types help clear complement-coated pathogens. (wikidoc.org)
Components10
- Third, the terminal complement components damage certain bacteria by creating pores in the bacterial membrane. (nih.gov)
- These cutting-edge techniques are suitable both for the basic scientist interested in understanding complement's mechanisms of activation and for the clinical scientist wishing to quantify its activation, and range from the purification of its components to generating complement-deficient mice by gene deletion. (springer.com)
- Complement Systems: Methods and Protocols is composed of 32 individual chapters that describe a variety of protocols to purify and analyze the activity of the individual complement components or pathways. (springer.com)
- 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 most commonly measured complement components. (medlineplus.gov)
- The model is concordant with the stability of the C1 complex during activation of the next complement components. (elsevier.com)
- We found that CRP was required for fixation of complement components C1q, C1r, C1s and MASP1. (nature.com)
- various complement components may be deficient without serious effects on the host. (thefreedictionary.com)
- Human complement components C1 and C1q (Sigma, St. Louis, Mo. (asm.org)
- More often, you will have lowered levels of several complement components at once. (rochester.edu)
Recombinant2
- A cheaper C1-inh alternative, recombinant C1-inh was also developed. (bionity.com)
- In direct ELISAs, less than 1% cross-reactivity with recombinant human Complement Component C1s is observed. (novusbio.com)
Regulator2
- Vag8 is a complement evasion molecule that mediates its effects by binding to the complement regulator C1 inhibitor (C1-INH). (uu.nl)
- Expression of Complement Regulator Genes in Aβ1-42 Stimulated Human Neuroblastoma Cell. (freepatentsonline.com)
Receptors1
- Under physiological conditions, opsonization of the crystals with complement or other opsonins may also eliminate the need for specific crystal receptors. (nature.com)
Activate2
- These findings indicate that SBA which are known to bind to a variety of oral microorganisms are also able to bind and activate the first complement component . (musc.edu)
- There was a direct correlation between the concentration of the guinea pig C1 inhibitor and the loss of ability of the PS to activate C1 in mixtures of constant concentrations of purified guinea pig C1 and purified venom PS, and increasing concentrations of purified guinea pig C1 inhibitor. (elsevier.com)
Component of complement1
- Immunofluorescence studies on the subcomponents of the first component of complement (C1): detection of C1q and C1s in different cells of biopsy material and on human as well as on guinea pig peritoneal macrophages. (freepatentsonline.com)
Infection3
- In this context, the complement, as one of the first line of host defense against infection was shown to play an important role in recognizing T. cruzi metacyclic trypomastigotes and in controlling parasite invasion. (frontiersin.org)
- An important step during T. cruzi infection is when intracellular trypomastigotes are release to bloodstream where they may be target by complement. (frontiersin.org)
- Your complement levels will often shoot up dramatically just after an infection or injury. (rochester.edu)
Complex5
- It is also emerging as an important factor in atherosclerosis: complement activation, and formation of the terminal C5b-9 membrane attack complex of complement were shown to occur in atherosclerotic lesions. (europa.eu)
- Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. (uniprot.org)
- C1s is part of the C1 complex . (wikipedia.org)
- Inhibition of Immune Complex Complement Activation And Neutrophil Extracellular. (realtalifesciences.com)
- A fluid phase form of the MAC, called the Terminal Complement Complex (TCC) can be found in the circulation after complement activation occurs and makes a useful laboratory marker for complement activation. (primaryimmune.org)
Inhibits Complement Activation1
- It inhibits complement activation at the yeast surface and, in addition, mediates adhesion of C. albicans to host endothelial cells. (labome.org)
20162
- 2016) How Dextran Sulfate Affects C1-inhibitor Activity: A Model for Polysaccharide Potentiation. (omicsonline.org)
- Risitano AM, Marotta S (2016) Therapeutic complement inhibition in complement-mediated hemolytic anemias: Past, present and future. (omicsonline.org)
Inflammatory8
- It is tenable that the increase in α1 antitrypsin and C1-INH (anti-inflammatory mediators) at T2 served to attenuate the acute phase/inflammatory response. (edu.au)
- Vascular endothelial cells and complement activation are thought to be involved in the inflammatory reactions following reperfusion, so endothelial targeting of complement inhibitors is of great interest. (elsevier.com)
- It becomes evident that excessive activation or insufficient control of complement activation on host cells can cause an immune imbalance that may fuel a vicious cycle between complement, inflammatory cells, and tissue damage that exacerbates clinical complications. (jimmunol.org)
- Although the exact involvement of complement needs to be carefully investigated for each disease, therapeutic modulation of complement activity emerges as an attractive target for upstream inhibition of inflammatory processes. (jimmunol.org)
- This review provides an update about the functional and collaborative capabilities of complement, highlights major disease areas with known complement contribution, and indicates the potential for complement as a focal point in immunomodulatory strategies for treating inflammatory diseases. (jimmunol.org)
- In the worst case, a vicious cycle between tissue damage, complement activation, and immune attack perpetually re-creates inflammatory stimulators rather than resolving them. (jimmunol.org)
- In view of this upstream position in inflammatory homeostasis, there is growing interest in understanding the role of complement in pathological processes and in exploiting complement targets for therapeutic modulation ( 3 , 4 ). (jimmunol.org)
- Fortunately, our knowledge about the functions of complement in health and disease has much improved, and new discoveries have revealed a fascinating cross-talk network that ties complement closely into the immune-inflammatory network ( 1 , 5 ). (jimmunol.org)
Gene3
- This gene is a member of the regulators of complement activation (RCA) family and is located in the 'cluster RCA' region of chromosome 1. (wikidoc.org)
- [3] In mice, CR1 is an alternatively spliced variant of the complement receptor 2 (CR2) gene. (wikidoc.org)
- Low complement C4B gene copy number predicts short-term mortality after acute myocardial infarction. (labome.org)
Inhibition of Complement Activation1
- Inhibition of Complement Activation, Myeloperoxidase, NET Formation And Oxidant. (realtalifesciences.com)
Subcomponent2
- The catalytic chain of human complement subcomponent C1̅. (biochemj.org)
- 1. The a- and b-chains of reduced and alkylated human complement subcomponent C1r were separated by high-pressure gel-permeation chromatography and isolated in good yield and in pure form. (biochemj.org)
Lysis3
- Under these conditions , peptides 98 and 102 caused complement-mediated hemo~sis , while none of the other peptides tested were capable o~ inducing such lysis . (musc.edu)
- Once activated, several relevant complement functions are generated which include opsonization and phagocytosis of particles or microorganisms and cell lysis. (frontiersin.org)
- Nevertheless, the parasite uses a sequence of events in order to escape from complement-mediated lysis. (frontiersin.org)
Human complement component3
- Molecular cloning of cDNA for human complement component C1s. (wikipedia.org)
- Human complement component C1s. (wikipedia.org)
- Detects human Complement Component C1r in direct ELISAs and Western blots. (novusbio.com)
Inhibited complement activation1
- After intravenous injection in rats, the pegylated derivative inhibited complement activation in the blood by 90% after 30 seconds, demonstrating extremely rapid function. (nih.gov)
Mechanism1
- Classical enzyme kinetic analyses were applied to define the mechanism of the effect of the fourth component (C4) on cleavage of the second component (C2) by the first component (C1) of human complement. (jimmunol.org)
Biological2
- In this review, we aim to present an overview on the strategies used by T. cruzi in order to circumvent the activation of complement and, consequently, its biological effects. (frontiersin.org)
- Many of the biological consequences of complement activation also await elucidation. (indigo.ca)
Inhibitors1
- Risitano AM (2015) Current and future pharmacologic complement inhibitors. (omicsonline.org)
Activation in vitro1
- Together these results show that PIC1 rapidly inhibits classical complement activation in vitro and in vivo and is functional for a variety of animal species, suggesting its utility in animal models of classical complement-mediated diseases. (nih.gov)
Functional1
- Both antigenic and functional C1 inhibitor levels will be determined. (clinicaltrials.gov)
Peptides1
- It would appear then that the area of IgG required for complement-mediated hemolysis of sensitized cells could be the region with a primary sequence similar to these peptides . (musc.edu)
Hemolytic2
- We further analyzed the effect of arginine and glutamic acid residue substitution on the complement inhibitory activity of our lead derivative in a hemolytic assay and found that the original sequence demonstrated superior inhibitory activity. (nih.gov)
- HAstV-1 virions and purified CP both suppress hemolytic complement activity. (realtalifesciences.com)
Substrates1
- In addition, our findings support the hypothesis that there are separate enzymatic sites on the C1 molecule for its natural substrates. (jimmunol.org)
Deposition2
Role of complement1
- Comprehensive and cutting-edge, Complement Methods and Protocols offers today's basic and clinical investigators powerful tools for the analysis of the role of complement in human pathophysiology and disease, as well as its therapeutic regulation. (springer.com)
Indicate that complement1
- Decades of basic science and clinical research indicate that complement factors are involved in septic shock. (hindawi.com)
Cleavage1
- C2 cleavage by C1 followed first order kinetics. (jimmunol.org)
Bind2
- These fluid phase complexes do not bind to cell membranes and are ultimately scavenged by clusterin and vitronectin, two regulators of complement. (wikipedia.org)
- This is useful if the binding agent is intended to bind both an effector cell and a target to be destroyed, because the effector cell can be protected from significant cell damage that might otherwise occur (e.g. due to premature activation of complement and/or ADCC). (google.com)
Activity7
- Complement testing may also be ordered occasionally when a health practitioner wants to monitor the current activity of a condition. (labcorp.com)
- Moreover, T. cruzi promotes secretion of plasma membrane-derived vesicles from host cells, which prevent the activity of C3 convertase C4b2a and thereby may hinder complement. (frontiersin.org)
- Although named after its complement inhibitory activity, C1-inh also inhibits proteinases of the fibrinolytic, clotting, and kinin pathways. (bionity.com)
- The serpin domain is similar to other serpins, and this part of C1-inh provides the inhibitory activity of C1-inh. (bionity.com)
- C4d complement split product in diagnosis of immunological activity of chronic allograft nephropathy. (labome.org)
- Your healthcare provider may also order a total complement activity test, or CH50. (rochester.edu)
- If the C4 test is done as part of a total complement activity test, the test should be repeated if low levels are found. (rochester.edu)