Complement c1. Medical search

A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.

A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B.

The smaller fragment formed when complement C4 is cleaved by COMPLEMENT C1S. It is an anaphylatoxin that causes symptoms of immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE) but its activity is weaker than that of COMPLEMENT C3A or COMPLEMENT C5A.

The 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.

A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.

The 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.

The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.

The large fragment formed when COMPLEMENT C4 is cleaved by COMPLEMENT C1S. The membrane-bound C4b binds COMPLEMENT C2A, a SERINE PROTEASE, to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.

The 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.

Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).

A 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.

A 206-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c (749-954), and C3dg (955-1303) in the presence COMPLEMENT FACTOR H.

A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).

A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

A 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.

Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.

A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).

A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.

A 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.

Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.

A 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.

Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.

A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.

Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.

Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.

A 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.

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.

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.

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).

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.

The COOH-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S. It is a SERINE PROTEASE. C2a combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).

A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.

Enzymes that activate one or more COMPLEMENT PROTEINS in the complement system leading to the formation of the COMPLEMENT MEMBRANE ATTACK COMPLEX, an important response in host defense. They are enzymes in the various COMPLEMENT ACTIVATION pathways.

Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.

A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.

Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.

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.

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.

Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.

A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.

A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.

A serum protein that regulates the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It binds as a cofactor to COMPLEMENT FACTOR I which then hydrolyzes the COMPLEMENT C4B in the CLASSICAL PATHWAY C3 CONVERTASE (C4bC2a).

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.

GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.

Important enzymes in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.

The N-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S.

Small glycoproteins found on both hematopoietic and non-hematopoietic cells. CD59 restricts the cytolytic activity of homologous complement by binding to C8 and C9 and blocking the assembly of the membrane attack complex. (From Barclay et al., The Leukocyte Antigen FactsBook, 1993, p234)

Venoms from snakes of the genus Naja (family Elapidae). They contain many specific proteins that have cytotoxic, hemolytic, neurotoxic, and other properties. Like other elapid venoms, they are rich in enzymes. They include cobramines and cobralysins.

The complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes IMMUNE COMPLEX DISEASES.

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).

Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.

An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.

The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.

The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.

A serine protease that is the complex of COMPLEMENT C3B and COMPLEMENT FACTOR BB. It cleaves multiple COMPLEMENT C3 into COMPLEMENT C3A (anaphylatoxin) and COMPLEMENT C3B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY.

A serine protease that cleaves multiple COMPLEMENT 5 into COMPLEMENT 5A (anaphylatoxin) and COMPLEMENT 5B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of CLASSICAL PATHWAY C3 CONVERTASE (C4b2a) with an additional COMPLEMENT C3B, or C4b2a3b.

Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.

A serine protease that cleaves multiple COMPLEMENT 3 into COMPLEMENT 3A (anaphylatoxin) and COMPLEMENT 3B in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. It is a complex of COMPLEMENT 4B and COMPLEMENT 2A (C4b2a).

A ubiquitously expressed complement receptor that binds COMPLEMENT C3B and COMPLEMENT C4B and serves as a cofactor for their inactivation. CD46 also interacts with a wide variety of pathogens and mediates immune response.

Proteins that bind to particles and cells to increase susceptibility to PHAGOCYTOSIS, especially ANTIBODIES bound to EPITOPES that attach to FC RECEPTORS. COMPLEMENT C3B may also participate.

Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.

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.

A serine protease that cleaves multiple COMPLEMENT C5 into COMPLEMENT C5A (anaphylatoxin) and COMPLEMENT C5B in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. It is the complex of ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) with an additional COMPLEMENT C3B, or C3bBb3b.

The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).

The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.

Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.

A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.

A derivative of complement C5a, generated when the carboxy-terminal ARGININE is removed by CARBOXYPEPTIDASE B present in normal human serum. C5a des-Arg shows complete loss of spasmogenic activity though it retains some chemotactic ability (CHEMOATTRACTANTS).

An adhesion-promoting leukocyte surface membrane heterodimer. The alpha subunit consists of the CD11b ANTIGEN and the beta subunit the CD18 ANTIGEN. The antigen, which is an integrin, functions both as a receptor for complement 3 and in cell-cell and cell-substrate adhesive interactions.

The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.

Granular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.

The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.

A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.

The clear portion of BLOOD that is left after BLOOD COAGULATION to remove BLOOD CELLS and clotting proteins.

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.

A class of immunoglobulin bearing mu chains (IMMUNOGLOBULIN MU-CHAINS). IgM can fix COMPLEMENT. The name comes from its high molecular weight and originally being called a macroglobulin.

A genus of trematode flukes belonging to the family Schistosomatidae. There are over a dozen species. These parasites are found in man and other mammals. Snails are the intermediate hosts.

A test used to determine whether or not complementation (compensation in the form of dominance) will occur in a cell with a given mutant phenotype when another mutant genome, encoding the same mutant phenotype, is introduced into that cell.

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.

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.

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.

Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.

Antibodies produced by a single clone of cells.

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.

Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.

Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.

Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.

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.

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.)

Established cell cultures that have the potential to propagate indefinitely.

The capacity of a normal organism to remain unaffected by microorganisms and their toxins. It results from the presence of naturally occurring ANTI-INFECTIVE AGENTS, constitutional factors such as BODY TEMPERATURE and immediate acting immune cells such as NATURAL KILLER CELLS.

Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.

Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.

The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.

Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.

The insertion of recombinant DNA molecules from prokaryotic and/or eukaryotic sources into a replicating vehicle, such as a plasmid or virus vector, and the introduction of the resultant hybrid molecules into recipient cells without altering the viability of those cells.

The parts of a macromolecule that directly participate in its specific combination with another molecule.

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.

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 in which a polyacrylamide gel is used as the diffusion medium.

A specific mannose-binding member of the collectin family of lectins. It binds to carbohydrate groups on invading pathogens and plays a key role in the MANNOSE-BINDING LECTIN COMPLEMENT PATHWAY.

Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.

Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).

Proteins prepared by recombinant DNA technology.

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.

Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.

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.

Plasma glycoproteins that form a stable complex with hemoglobin to aid the recycling of heme iron. They are encoded in man by a gene on the short arm of chromosome 16.

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).

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 whose amino and carboxy ends are linked together with a peptide bond forming a circular chain. Some of them are ANTI-INFECTIVE AGENTS. Some of them are biosynthesized non-ribosomally (PEPTIDE BIOSYNTHESIS, NON-RIBOSOMAL).

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).

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.

The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.

Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.

Plasmids containing at least one cos (cohesive-end site) of PHAGE LAMBDA. They are used as cloning vehicles.

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.

Proteins found in any species of bacterium.

Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.

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.

A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.

Transport proteins that carry specific substances in the blood or across cell membranes.

Serum serine proteases which participate in COMPLEMENT ACTIVATION. They are activated when complexed with the MANNOSE-BINDING LECTIN, therefore also known as Mannose-binding protein-Associated Serine Proteases (MASPs). They cleave COMPLEMENT C4 and COMPLEMENT C2 to form C4b2a, the CLASSICAL PATHWAY C3 CONVERTASE.

A 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.

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.

An individual in which both alleles at a given locus are identical.

Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.

The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.

Biologically active substances whose activities affect or play a role in the functioning of the immune system.

Elements of limited time intervals, contributing to particular results or situations.

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.

Histochemical localization of immunoreactive substances using labeled antibodies as reagents.

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.

The genetic constitution of individuals with respect to one member of a pair of allelic genes, or sets of genes that are closely linked and tend to be inherited together such as those of the MAJOR HISTOCOMPATIBILITY COMPLEX.

Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.

Antigens determined by leukocyte loci found on chromosome 6, the major histocompatibility loci in humans. They are polypeptides or glycoproteins found on most nucleated cells and platelets, determine tissue types for transplantation, and are associated with certain diseases.

Glycoproteins found on the membrane or surface of cells.

The sequential correspondence of nucleotides in one nucleic acid molecule with those of another nucleic acid molecule. Sequence homology is an indication of the genetic relatedness of different organisms and gene function.

The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.

Large, phagocytic mononuclear leukocytes produced in the vertebrate BONE MARROW and released into the BLOOD; contain a large, oval or somewhat indented nucleus surrounded by voluminous cytoplasm and numerous organelles.

The sum of the weight of all the atoms in a molecule.

The rate dynamics in chemical or physical systems.

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.

The parts of a transcript of a split GENE remaining after the INTRONS are removed. They are spliced together to become a MESSENGER RNA or other functional RNA.

Lymphoid cells concerned with humoral immunity. They are short-lived cells resembling bursa-derived lymphocytes of birds in their production of immunoglobulin upon appropriate stimulation.

The presence of proteins in the urine, an indicator of KIDNEY DISEASES.

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.

The production of ANTIBODIES by proliferating and differentiated B-LYMPHOCYTES under stimulation by ANTIGENS.

Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.

A gram-positive organism found in the upper respiratory tract, inflammatory exudates, and various body fluids of normal and/or diseased humans and, rarely, domestic animals.

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.

Use of restriction endonucleases to analyze and generate a physical map of genomes, genes, or other segments of DNA.

A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms.

Short sequences (generally about 10 base pairs) of DNA that are complementary to sequences of messenger RNA and allow reverse transcriptases to start copying the adjacent sequences of mRNA. Primers are used extensively in genetic and molecular biology techniques.

A plasma protein that circulates in increased amounts during inflammation and after tissue damage.

The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.

A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.

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)

Cytochrome P-450 monooxygenases (MIXED FUNCTION OXYGENASES) that are important in steroid biosynthesis and metabolism.

Detection of RNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.

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.

Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.

A method (first developed by E.M. Southern) for detection of DNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.

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.

Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.

A constitution or condition of the body which makes the tissues react in special ways to certain extrinsic stimuli and thus tends to make the individual more than usually susceptible to certain diseases.

Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.

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.

A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.

The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells.

The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.

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.

Variation occurring within a species in the presence or length of DNA fragment generated by a specific endonuclease at a specific site in the genome. Such variations are generated by mutations that create or abolish recognition sites for these enzymes or change the length of the fragment.

The proportion of one particular in the total of all ALLELES for one genetic locus in a breeding POPULATION.

A common name used for the genus Cavia. The most common species is Cavia porcellus which is the domesticated guinea pig used for pets and biomedical research.

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.

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.

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.

Potentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.

The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.

A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.

Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood.

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.

Immunoglobulins produced in a response to BACTERIAL ANTIGENS.

The systematic study of the complete complement of proteins (PROTEOME) of organisms.

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.

A cytokine that stimulates the growth and differentiation of B-LYMPHOCYTES and is also a growth factor for HYBRIDOMAS and plasmacytomas. It is produced by many different cells including T-LYMPHOCYTES; MONOCYTES; and FIBROBLASTS.

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).

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.

The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.

A large collection of DNA fragments cloned (CLONING, MOLECULAR) from a given organism, tissue, organ, or cell type. It may contain complete genomic sequences (GENOMIC LIBRARY) or complementary DNA sequences, the latter being formed from messenger RNA and lacking intron sequences.

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.

A latent susceptibility to disease at the genetic level, which may be activated under certain conditions.

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.

A single nucleotide variation in a genetic sequence that occurs at appreciable frequency in the population.

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)

There are two main types of hemolysis:

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

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

Some common causes of hemolysis include:

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

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

The term "systemic" refers to the fact that the disease affects multiple organ systems, including the skin, joints, kidneys, lungs, and nervous system. LES is a complex condition, and its symptoms can vary widely depending on which organs are affected. Common symptoms include fatigue, fever, joint pain, rashes, and swelling in the extremities.

There are several subtypes of LES, including:

1. Systemic lupus erythematosus (SLE): This is the most common form of the disease, and it can affect anyone, regardless of age or gender.
2. Discoid lupus erythematosus (DLE): This subtype typically affects the skin, causing a red, scaly rash that does not go away.
3. Drug-induced lupus erythematosus: This form of the disease is caused by certain medications, and it usually resolves once the medication is stopped.
4. Neonatal lupus erythematosus: This rare condition affects newborn babies of mothers with SLE, and it can cause liver and heart problems.

There is no cure for LES, but treatment options are available to manage the symptoms and prevent flares. Treatment may include nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, immunosuppressive medications, and antimalarial drugs. In severe cases, hospitalization may be necessary to monitor and treat the disease.

It is important for people with LES to work closely with their healthcare providers to manage their condition and prevent complications. With proper treatment and self-care, many people with LES can lead active and fulfilling lives.

Idiopathic membranous nephropathy (IMN) is an autoimmune disorder that causes GNM without any identifiable cause. Secondary membranous nephropathy, on the other hand, is caused by systemic diseases such as lupus or cancer.

The symptoms of GNM can vary depending on the severity of the disease and may include blood in the urine, proteinuria, edema, high blood pressure, and decreased kidney function. The diagnosis of GNM is based on a combination of clinical findings, laboratory tests, and renal biopsy.

Treatment for GNM is aimed at slowing the progression of the disease and managing symptoms. Medications such as corticosteroids, immunosuppressive drugs, and blood pressure-lowering drugs may be used to treat GNM. In some cases, kidney transplantation may be necessary.

The prognosis for GNM varies depending on the severity of the disease and the underlying cause. In general, the prognosis for IMN is better than for secondary membranous nephropathy. With proper treatment, some patients with GNM can experience a slowing or stabilization of the disease, while others may progress to end-stage renal disease (ESRD).

The cause of GNM is not fully understood, but it is believed to be an autoimmune disorder that leads to inflammation and damage to the glomerular membrane. Genetic factors and environmental triggers may also play a role in the development of GNM.

There are several risk factors for developing GNM, including family history, age (GMN is more common in adults), and certain medical conditions such as hypertension and diabetes.

The main complications of GNM include:

1. ESRD: Progression to ESRD is a common outcome of untreated GNM.
2. High blood pressure: GNM can lead to high blood pressure, which can further damage the kidneys.
3. Infections: GNM increases the risk of infections due to impaired immune function.
4. Kidney failure: GNM can cause chronic kidney failure, leading to the need for dialysis or a kidney transplant.
5. Cardiovascular disease: GNM is associated with an increased risk of cardiovascular disease, including heart attack and stroke.
6. Malnutrition: GNM can lead to malnutrition due to decreased appetite, nausea, and vomiting.
7. Bone disease: GNM can cause bone disease, including osteoporosis and bone pain.
8. Anemia: GNM can cause anemia, which can lead to fatigue, weakness, and shortness of breath.
9. Increased risk of infections: GNM increases the risk of infections due to impaired immune function.
10. Decreased quality of life: GNM can significantly decrease a person's quality of life, leading to decreased mobility, pain, and discomfort.

It is important for individuals with GNM to receive early diagnosis and appropriate treatment to prevent or delay the progression of these complications.

The symptoms of glomerulonephritis can vary depending on the underlying cause of the disease, but may include:

* Blood in the urine (hematuria)
* Proteinuria (excess protein in the urine)
* Reduced kidney function
* Swelling in the legs and ankles (edema)
* High blood pressure

Glomerulonephritis can be caused by a variety of factors, including:

* Infections such as staphylococcal or streptococcal infections
* Autoimmune disorders such as lupus or rheumatoid arthritis
* Allergic reactions to certain medications
* Genetic defects
* Certain diseases such as diabetes, high blood pressure, and sickle cell anemia

The diagnosis of glomerulonephritis typically involves a physical examination, medical history, and laboratory tests such as urinalysis, blood tests, and kidney biopsy.

Treatment for glomerulonephritis depends on the underlying cause of the disease and may include:

* Antibiotics to treat infections
* Medications to reduce inflammation and swelling
* Diuretics to reduce fluid buildup in the body
* Immunosuppressive medications to suppress the immune system in cases of autoimmune disorders
* Dialysis in severe cases

The prognosis for glomerulonephritis depends on the underlying cause of the disease and the severity of the inflammation. In some cases, the disease may progress to end-stage renal disease, which requires dialysis or a kidney transplant. With proper treatment, however, many people with glomerulonephritis can experience a good outcome and maintain their kidney function over time.

Arteriolosclerosis is often associated with conditions such as hypertension, diabetes, and atherosclerosis, which is the buildup of plaque in the arteries. It can also be caused by other factors such as smoking, high cholesterol levels, and inflammation.

The symptoms of arteriolosclerosis can vary depending on the location and severity of the condition, but may include:

* Decreased blood flow to organs or tissues
* Fatigue
* Weakness
* Shortness of breath
* Dizziness or lightheadedness
* Pain in the affected limbs or organs

Arteriolosclerosis is typically diagnosed through a combination of physical examination, medical history, and diagnostic tests such as ultrasound, angiography, or blood tests. Treatment for the condition may include lifestyle changes such as exercise and dietary modifications, medications to control risk factors such as hypertension and high cholesterol, and in some cases, surgical intervention to open or bypass blocked arterioles.

In summary, arteriolosclerosis is a condition where the arterioles become narrowed or obstructed, leading to decreased blood flow to organs and tissues and potentially causing a range of health problems. It is often associated with other conditions such as hypertension and atherosclerosis, and can be diagnosed through a combination of physical examination, medical history, and diagnostic tests. Treatment may include lifestyle changes and medications to control risk factors, as well as surgical intervention in some cases.

1) They share similarities with humans: Many animal species share similar biological and physiological characteristics with humans, making them useful for studying human diseases. For example, mice and rats are often used to study diseases such as diabetes, heart disease, and cancer because they have similar metabolic and cardiovascular systems to humans.

2) They can be genetically manipulated: Animal disease models can be genetically engineered to develop specific diseases or to model human genetic disorders. This allows researchers to study the progression of the disease and test potential treatments in a controlled environment.

3) They can be used to test drugs and therapies: Before new drugs or therapies are tested in humans, they are often first tested in animal models of disease. This allows researchers to assess the safety and efficacy of the treatment before moving on to human clinical trials.

4) They can provide insights into disease mechanisms: Studying disease models in animals can provide valuable insights into the underlying mechanisms of a particular disease. This information can then be used to develop new treatments or improve existing ones.

5) Reduces the need for human testing: Using animal disease models reduces the need for human testing, which can be time-consuming, expensive, and ethically challenging. However, it is important to note that animal models are not perfect substitutes for human subjects, and results obtained from animal studies may not always translate to humans.

6) They can be used to study infectious diseases: Animal disease models can be used to study infectious diseases such as HIV, TB, and malaria. These models allow researchers to understand how the disease is transmitted, how it progresses, and how it responds to treatment.

7) They can be used to study complex diseases: Animal disease models can be used to study complex diseases such as cancer, diabetes, and heart disease. These models allow researchers to understand the underlying mechanisms of the disease and test potential treatments.

8) They are cost-effective: Animal disease models are often less expensive than human clinical trials, making them a cost-effective way to conduct research.

9) They can be used to study drug delivery: Animal disease models can be used to study drug delivery and pharmacokinetics, which is important for developing new drugs and drug delivery systems.

10) They can be used to study aging: Animal disease models can be used to study the aging process and age-related diseases such as Alzheimer's and Parkinson's. This allows researchers to understand how aging contributes to disease and develop potential treatments.

There are several types of lupus nephritis, each with its own unique characteristics and symptoms. The most common forms include:

* Class I (mesangial proliferative glomerulonephritis): This type is characterized by the growth of abnormal cells in the glomeruli (blood-filtering units of the kidneys).
* Class II (active lupus nephritis): This type is characterized by widespread inflammation and damage to the kidneys, with or without the presence of antibodies.
* Class III (focal lupus nephritis): This type is characterized by localized inflammation in certain areas of the kidneys.
* Class IV (lupus nephritis with crescentic glomerulonephritis): This type is characterized by widespread inflammation and damage to the kidneys, with crescent-shaped tissue growth in the glomeruli.
* Class V (lupus nephritis with sclerotic changes): This type is characterized by hardening and shrinkage of the glomeruli due to scarring.

Lupus Nephritis can cause a range of symptoms, including:

* Proteinuria (excess protein in the urine)
* Hematuria (blood in the urine)
* Reduced kidney function
* Swelling (edema)
* Fatigue
* Fever
* Joint pain

Lupus Nephritis can be diagnosed through a combination of physical examination, medical history, laboratory tests, and kidney biopsy. Treatment options for lupus nephritis include medications to suppress the immune system, control inflammation, and prevent further damage to the kidneys. In severe cases, dialysis or a kidney transplant may be necessary.

There are several key features of inflammation:

1. Increased blood flow: Blood vessels in the affected area dilate, allowing more blood to flow into the tissue and bringing with it immune cells, nutrients, and other signaling molecules.
2. Leukocyte migration: White blood cells, such as neutrophils and monocytes, migrate towards the site of inflammation in response to chemical signals.
3. Release of mediators: Inflammatory mediators, such as cytokines and chemokines, are released by immune cells and other cells in the affected tissue. These molecules help to coordinate the immune response and attract more immune cells to the site of inflammation.
4. Activation of immune cells: Immune cells, such as macrophages and T cells, become activated and start to phagocytose (engulf) pathogens or damaged tissue.
5. Increased heat production: Inflammation can cause an increase in metabolic activity in the affected tissue, leading to increased heat production.
6. Redness and swelling: Increased blood flow and leakiness of blood vessels can cause redness and swelling in the affected area.
7. Pain: Inflammation can cause pain through the activation of nociceptors (pain-sensing neurons) and the release of pro-inflammatory mediators.

Inflammation can be acute or chronic. Acute inflammation is a short-term response to injury or infection, which helps to resolve the issue quickly. Chronic inflammation is a long-term response that can cause ongoing damage and diseases such as arthritis, asthma, and cancer.

There are several types of inflammation, including:

1. Acute inflammation: A short-term response to injury or infection.
2. Chronic inflammation: A long-term response that can cause ongoing damage and diseases.
3. Autoimmune inflammation: An inappropriate immune response against the body's own tissues.
4. Allergic inflammation: An immune response to a harmless substance, such as pollen or dust mites.
5. Parasitic inflammation: An immune response to parasites, such as worms or fungi.
6. Bacterial inflammation: An immune response to bacteria.
7. Viral inflammation: An immune response to viruses.
8. Fungal inflammation: An immune response to fungi.

There are several ways to reduce inflammation, including:

1. Medications such as nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids, and disease-modifying anti-rheumatic drugs (DMARDs).
2. Lifestyle changes, such as a healthy diet, regular exercise, stress management, and getting enough sleep.
3. Alternative therapies, such as acupuncture, herbal supplements, and mind-body practices.
4. Addressing underlying conditions, such as hormonal imbalances, gut health issues, and chronic infections.
5. Using anti-inflammatory compounds found in certain foods, such as omega-3 fatty acids, turmeric, and ginger.

It's important to note that chronic inflammation can lead to a range of health problems, including:

1. Arthritis
2. Diabetes
3. Heart disease
4. Cancer
5. Alzheimer's disease
6. Parkinson's disease
7. Autoimmune disorders, such as lupus and rheumatoid arthritis.

Therefore, it's important to manage inflammation effectively to prevent these complications and improve overall health and well-being.

There are three main forms of ACH:

1. Classic congenital adrenal hyperplasia (CAH): This is the most common form of ACH, accounting for about 90% of cases. It is caused by mutations in the CYP21 gene, which codes for an enzyme that converts cholesterol into cortisol and aldosterone.
2. Non-classic CAH (NCAH): This form of ACH is less common than classic CAH and is caused by mutations in other genes involved in cortisol and aldosterone production.
3. Mineralocorticoid excess (MOE) or glucocorticoid deficiency (GD): These are rare forms of ACH that are characterized by excessive production of mineralocorticoids (such as aldosterone) or a deficiency of glucocorticoids (such as cortisol).

The symptoms of ACH can vary depending on the specific form of the disorder and the age at which it is diagnosed. In classic CAH, symptoms typically appear in infancy and may include:

* Premature puberty (in girls) or delayed puberty (in boys)
* Abnormal growth patterns
* Distended abdomen
* Fatigue
* Weight gain or obesity
* Easy bruising or bleeding

In NCAH and MOE/GD, symptoms may be less severe or may not appear until later in childhood or adulthood. They may include:

* High blood pressure
* Low blood sugar (hypoglycemia)
* Weight gain or obesity
* Fatigue
* Mood changes

If left untreated, ACH can lead to serious complications, including:

* Adrenal gland insufficiency
* Heart problems
* Bone health problems
* Increased risk of infections
* Mental health issues (such as depression or anxiety)

Treatment for ACH typically involves hormone replacement therapy to restore the balance of hormones in the body. This may involve taking medications such as cortisol, aldosterone, or other hormones to replace those that are deficient or imbalanced. In some cases, surgery may be necessary to remove an adrenal tumor or to correct physical abnormalities.

With proper treatment, many individuals with ACH can lead healthy, active lives. However, it is important for individuals with ACH to work closely with their healthcare providers to manage their condition and prevent complications. This may involve regular check-ups, hormone level monitoring, and lifestyle changes such as a healthy diet and regular exercise.

Proteinuria is usually diagnosed by a urine protein-to-creatinine ratio (P/C ratio) or a 24-hour urine protein collection. The amount and duration of proteinuria can help distinguish between different underlying causes and predict prognosis.

Proteinuria can have significant clinical implications, as it is associated with increased risk of cardiovascular disease, kidney damage, and malnutrition. Treatment of the underlying cause can help reduce or eliminate proteinuria.

There are two main types of MD:

1. Dry Macular Degeneration (DMD): This is the most common form of MD, accounting for about 90% of cases. It is caused by the gradual accumulation of waste material in the macula, which can lead to cell death and vision loss over time.
2. Wet Macular Degeneration (WMD): This type of MD is less common but more aggressive, accounting for about 10% of cases. It occurs when new blood vessels grow underneath the retina, leaking fluid and causing damage to the macula. This can lead to rapid vision loss if left untreated.

The symptoms of MD can vary depending on the severity and type of the condition. Common symptoms include:

* Blurred vision
* Distorted vision (e.g., straight lines appearing wavy)
* Difficulty reading or recognizing faces
* Difficulty adjusting to bright light
* Blind spots in central vision

MD can have a significant impact on daily life, making it difficult to perform everyday tasks such as driving, reading, and recognizing faces.

There is currently no cure for MD, but there are several treatment options available to slow down the progression of the disease and manage its symptoms. These include:

* Anti-vascular endothelial growth factor (VEGF) injections: These medications can help prevent the growth of new blood vessels and reduce inflammation in the macula.
* Photodynamic therapy: This involves the use of a light-sensitive drug and low-intensity laser to damage and shrink the abnormal blood vessels in the macula.
* Vitamin supplements: Certain vitamins, such as vitamin C, E, and beta-carotene, have been shown to slow down the progression of MD.
* Laser surgery: This can be used to reduce the number of abnormal blood vessels in the macula and improve vision.

It is important for individuals with MD to receive regular monitoring and treatment from an eye care professional to manage their condition and prevent complications.

There are several types of disease susceptibility, including:

1. Genetic predisposition: This refers to the inherent tendency of an individual to develop a particular disease due to their genetic makeup. For example, some families may have a higher risk of developing certain diseases such as cancer or heart disease due to inherited genetic mutations.
2. Environmental susceptibility: This refers to the increased risk of developing a disease due to exposure to environmental factors such as pollutants, toxins, or infectious agents. For example, someone who lives in an area with high levels of air pollution may be more susceptible to developing respiratory problems.
3. Lifestyle susceptibility: This refers to the increased risk of developing a disease due to unhealthy lifestyle choices such as smoking, lack of exercise, or poor diet. For example, someone who smokes and is overweight may be more susceptible to developing heart disease or lung cancer.
4. Immune system susceptibility: This refers to the increased risk of developing a disease due to an impaired immune system. For example, people with autoimmune disorders such as HIV/AIDS or rheumatoid arthritis may be more susceptible to opportunistic infections.

Understanding disease susceptibility can help healthcare providers identify individuals who are at risk of developing certain diseases and provide preventive measures or early intervention to reduce the risk of disease progression. Additionally, genetic testing can help identify individuals with a high risk of developing certain diseases, allowing for earlier diagnosis and treatment.

In summary, disease susceptibility refers to the predisposition of an individual to develop a particular disease or condition due to various factors such as genetics, environment, lifestyle choices, and immune system function. Understanding disease susceptibility can help healthcare providers identify individuals at risk and provide appropriate preventive measures or early intervention to reduce the risk of disease progression.

There are several symptoms of RA, including:

1. Joint pain and stiffness, especially in the hands and feet
2. Swollen and warm joints
3. Redness and tenderness in the affected areas
4. Fatigue, fever, and loss of appetite
5. Loss of range of motion in the affected joints
6. Firm bumps of tissue under the skin (rheumatoid nodules)

RA can be diagnosed through a combination of physical examination, medical history, blood tests, and imaging studies such as X-rays or ultrasound. Treatment typically involves a combination of medications, including nonsteroidal anti-inflammatory drugs (NSAIDs), disease-modifying anti-rheumatic drugs (DMARDs), and biologic agents. Lifestyle modifications such as exercise and physical therapy can also be helpful in managing symptoms and improving quality of life.

There is no cure for RA, but early diagnosis and aggressive treatment can help to slow the progression of the disease and reduce symptoms. With proper management, many people with RA are able to lead active and fulfilling lives.

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

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

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

Examples of diseases with a known genetic predisposition:

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

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

The disorder is caused by mutations in the HBB gene that codes for the beta-globin subunit of hemoglobin. These mutations result in the production of abnormal hemoglobins that are unstable and prone to breakdown, leading to the release of free hemoglobin into the urine.

HP is classified into two types based on the severity of symptoms:

1. Type 1 HP: This is the most common form of the disorder and is characterized by mild to moderate anemia, occasional hemoglobinuria, and a normal life expectancy.
2. Type 2 HP: This is a more severe form of the disorder and is characterized by severe anemia, recurrent hemoglobinuria, and a shorter life expectancy.

There is no cure for HP, but treatment options are available to manage symptoms and prevent complications. These may include blood transfusions, folic acid supplements, and medications to reduce the frequency and severity of hemoglobinuria episodes.

The term "immune complex disease" was first used in the 1960s to describe a group of conditions that were thought to be caused by the formation of immune complexes. These diseases include:

1. Systemic lupus erythematosus (SLE): an autoimmune disorder that can affect multiple organ systems and is characterized by the presence of anti-nuclear antibodies.
2. Rheumatoid arthritis (RA): an autoimmune disease that causes inflammation in the joints and can lead to joint damage.
3. Type III hypersensitivity reaction: a condition in which immune complexes are deposited in tissues, leading to inflammation and tissue damage.
4. Pemphigus: a group of autoimmune diseases that affect the skin and mucous membranes, characterized by the presence of autoantibodies against desmosomal antigens.
5. Bullous pemphigoid: an autoimmune disease that affects the skin and is characterized by the formation of large blisters.
6. Myasthenia gravis: an autoimmune disorder that affects the nervous system, causing muscle weakness and fatigue.
7. Goodpasture's syndrome: a rare autoimmune disease that affects the kidneys and lungs, characterized by the presence of immune complexes in the glomeruli of the kidneys.
8. Hemolytic uremic syndrome (HUS): a condition in which red blood cells are destroyed and waste products accumulate in the kidneys, leading to kidney failure.

Immune complex diseases can be caused by various factors, including genetic predisposition, environmental triggers, and exposure to certain drugs or toxins. Treatment options for these diseases include medications that suppress the immune system, such as corticosteroids and immunosuppressive drugs, and plasmapheresis, which is a process that removes harmful antibodies from the blood. In some cases, organ transplantation may be necessary.

In conclusion, immune complex diseases are a group of disorders that occur when the body's immune system mistakenly attacks its own tissues and organs, leading to inflammation and damage. These diseases can affect various parts of the body, including the skin, kidneys, lungs, and nervous system. Treatment options vary depending on the specific disease and its severity, but may include medications that suppress the immune system and plasmapheresis.

A C1-INH concentrate can be used for angio-oedema (C1-INH deficiency). Pneumococcus and Haemophilus infections can be prevented ... "Complement Deficiencies. What are complement deficiencies?". patient.info. Retrieved 31 December 2017. "Complement Deficiencies ... C1-inhibitor deficiency or hereditary angioedema will have low C4 with normal C1 levels. Acquired hypocomplementemia may occur ... Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. ...

https://en.wikipedia.org/wiki/Complement_deficiency

... (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... C1q - another part of the C1 complex C1r - another part of the C1 complex MASP-2 - a protein similar to C1s, part of the lectin ... involved in the complement system. C1s is part of the C1 complex. In humans, it is encoded by the C1S gene. C1s cleaves C4 and ... "Entrez Gene: C1S Complement component 1, s subcomponent". Luo C, Thielens NM, Gagnon J, Gal P, Sarvari M, Tseng Y, Tosi M, ...

https://en.wikipedia.org/wiki/Complement_component_1s

This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named ... C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. ... C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is ... Inhibition of the complement cascade can decrease this damage. C1-inhibitor is contained in the human blood; it can, therefore ...

https://en.wikipedia.org/wiki/C1-inhibitor

"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. " ...

https://en.wikipedia.org/wiki/Ganeden

The classical pathway of complement activation is initiated when the C1 complex, made up of C1r and C1s serine proteases, ... 12th European Meeting on Complement in Human Disease12th European Meeting on CHD12th European Meeting on Complement in Human ... C3a is one of the proteins formed by the cleavage of complement component 3; the other is C3b. C3a is a 77 residue ... Anaphylatoxins are small complement peptides that induce proinflammatory responses in tissues. C3a is primarily regarded for ...

https://en.wikipedia.org/wiki/C3a_(complement)

C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity. C1- ... Alternative complement pathway - another complement system pathway Lectin pathway - another complement system pathway Noris, ... Obesity in turn results in an abnormally high level of complement activation via production of the C1 component of the ... Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema. ...

https://en.wikipedia.org/wiki/Classical_complement_pathway

Activation of the C1 complex initiates the classical complement pathway of the complement system. The antibodies IgM and all ... C1q is a subunit of the C1 enzyme complex that activates the serum complement system. C1q comprises 6 A, 6 B and 6 C chains. ... The complement component 1q (or simply C1q) is a protein complex involved in the complement system, which is part of the innate ... Deciphering the fine details of C1 assembly and activation mechanisms: "mission impossible"? - detailed diagrams Complement+C1q ...

https://en.wikipedia.org/wiki/Complement_component_1q

The classical pathway is inhibited by C1-inhibitor, which binds to C1 to prevent its activation. C3-convertase can be inhibited ... Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor ... The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability ... Three biochemical pathways activate the complement system: the classical complement pathway, the alternative complement pathway ...

https://en.wikipedia.org/wiki/Complement_system

The most useful complement studies obtained are as follows: C4 level C3 level C1 INH antigenic level C1 INH function C1q level ... The C1 esterase inhibitor (C1-INH) enzyme plays a role in the classical pathway of the complement cascade, which is a component ... The role of C1-INH is to regulate and control the activities of the complement cascade, such that complement proteins remain in ... including complement component 2 (C2), complement component 3 (C3), and complement component 4 (C4). When these particular ...

https://en.wikipedia.org/wiki/Acquired_C1_esterase_inhibitor_deficiency

Suppose its complement in the plane has countably many connected components C1, C2, C3, ... and suppose: the diameter of Ci ...

https://en.wikipedia.org/wiki/Sierpiński_carpet

C1 Inh) and the subcomponents C1r and C1s of the first component of complement, C1". The Biochemical Journal. 201 (1): 61-70. ... C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme ... Arlaud GJ, Gagnon J (April 1983). "Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r". ... Complement C1r subcomponent (EC 3.4.21.41, activated complement C1r, C overbar 1r esterase, C1r) is a protein involved in the ...

https://en.wikipedia.org/wiki/Complement_component_1r

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, ...

https://en.wikipedia.org/wiki/GraalVM

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 ...

https://en.wikipedia.org/wiki/C3b

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 ...

https://en.wikipedia.org/wiki/Angioedema

"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 ...

https://en.wikipedia.org/wiki/Albert_J._R._Heck

The CH50 is testing the classical complement pathway in an individual thus requiring functioning C1-C9 factors. If an ... Total complement activity (TCA) refers to a series of tests that determine the functioning of the complement system in an ... Decreased CH50 values may be seen in cirrhosis or hepatitis as a result of impaired complement production in the liver. It can ... For example, if and individual has normal C3/C4 values but a decreased CH50, that can indicate a terminal complement pathway ...

https://en.wikipedia.org/wiki/Total_complement_activity

With limited diffusion, there is a higher concentration of IgM, factor C1 of the complement cascade, and Langerhans cells. Any ... However, antigen-antibody complexes formed may deposit in the vascular endothelium and activate complements leading to severe ...

https://en.wikipedia.org/wiki/Peripheral_ulcerative_keratitis

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 ...

https://en.wikipedia.org/wiki/Polyclonal_antibodies

... 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 ...

https://en.wikipedia.org/wiki/Aggrecan

When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The ... cutaneous lupus mucinosis complement deficiency syndromes drug-induced lupus erythematosus neonatal lupus erythematosus ...

https://en.wikipedia.org/wiki/Lupus_erythematosus

... chondroitin sulfate proteoglycan of human B cell lines binds to the complement protein C1q and inhibits complex formation of C1 ...

https://en.wikipedia.org/wiki/CSPG4

"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, ...

https://en.wikipedia.org/wiki/Borrelia_burgdorferi

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 ... Complement component 1 Q subcomponent-binding protein, mitochondrial is a protein that in humans is encoded by the C1QBP gene. ... "Entrez Gene: C1QBP complement component 1, q subcomponent binding protein". Jiang, Jianzhong (1999). "rystal structure of human ...

https://en.wikipedia.org/wiki/C1QBP

... 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 ...

https://en.wikipedia.org/wiki/Urticarial_vasculitis

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. ...

https://en.wikipedia.org/wiki/Procollagen-proline_dioxygenase

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 ...

https://en.wikipedia.org/wiki/C1QA

The C1 complex (complement component 1, C1) is a protein complex involved in the complement system. It is the first component ... Deciphering the fine details of C1 assembly and activation mechanisms: "mission impossible"? Complement+C1 at the US National ... Activation of the C1 complex initiates the classical complement pathway. This occurs when C1q binds to antigen-antibody ... of the classical complement pathway and is composed of the subcomponents C1q, C1r and C1s. The C1 complex is composed of 1 ...

https://en.wikipedia.org/wiki/C1_complex

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 ...

https://en.wikipedia.org/wiki/Immunoglobulin_M

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. ...

https://en.wikipedia.org/wiki/Centro_Sismológico_Nacional

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 ...

https://en.wikipedia.org/wiki/Serpin

C1] to scientific and bibliographic representation of plants, animals and cities by Claudio Gay, Charles Thorold Wood and the ... painting was no longer regarded as a complement to other arts and science and gained its own value. Chilean art suffered ...

https://en.wikipedia.org/wiki/Chilean_art

It was intended for Group B sports car racing, complementing the purpose-built 956 and 962C which ran Group C in the World ... was also faster than several C1 and C2 class prototypes. At the race in June, Metge was joined by fellow Frenchman Claude ...

https://en.wikipedia.org/wiki/Porsche_961

C1-2. Retrieved 20 August 2015 - via newspapers.com. Ram Dass 1978, p. 27. Hari Dass 2019, p. 67. Hari Dass 2019, p. 27. ... Later, kirtan, mudras, fire ceremony (yajna), and satsang were introduced to complement a busy schedule. In 1972, as interest ...

https://en.wikipedia.org/wiki/Baba_Hari_Dass

Common cold Common mesentery Common variable immunodeficiency Compartment syndrome Complement component 2 deficiency Complement ... Alphabetical list 0-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z See also Health Exercise Nutrition C syndrome C1 ...

https://en.wikipedia.org/wiki/List_of_diseases_(C)

p. C1. ISSN 0362-4331. ProQuest 110319579. "New Sculpture at Fort Clinton Blends With the Shape of Its Surroundings". The New ... West Battery was intended to complement the three-tiered Castle Williams, the East Battery, on Governors Island. The fort was ... As part of this plan, an amphitheater would have been constructed in the southern end of Battery Park, complementing Castle ...

https://en.wikipedia.org/wiki/Castle_Clinton

In 1985, WYMT-TV was built as a complement to WKYT-TV. Financial troubles at Kentucky Central led to its takeover by the state ... p. C1, C10. Archived from the original on December 12, 2021. Retrieved December 12, 2021 - via Newspapers.com. "WDKY FOX 56 ...

https://en.wikipedia.org/wiki/WKYT-TV

p. C1. Retrieved January 1, 2012. Barmash, Jerry. "Marking New Year's Eve at Times Square Without Dick Clark". FishbowlNY. ... visual and digital offerings to complement whatever limited live entertainment or experiences-still in development-will take ...

https://en.wikipedia.org/wiki/Times_Square_Ball

"Records: Sub-class : C-1 (Landplanes) Group 3: turbo-jet." records.fai.org. Retrieved: 30 June 2011. Rich and Janos 1994, p. 85 ... Thus, there are doubts that the US has abandoned the concept of spy planes to complement reconnaissance satellites. Unmanned ...

https://en.wikipedia.org/wiki/Lockheed_SR-71_Blackbird

C1. Forrest, harriet, The Hampton Union (April 20, 2004). Sip & Sample Archived 2005-09-04 at the Wayback Machine Craig, Steve ... planted six grapevines in his backyard to complement his small garden and orchard. With time he concluded the microclimate of ...

https://en.wikipedia.org/wiki/Jewell_Towne_Vineyards

... so C1([0, 1]) ⊆ C([0, 1]). We claim that d/dx : C([0, 1]) → C([0, 1]) is a well-defined unbounded operator, with domain C1([0, ... coincides with the orthogonal complement of the range of the adjoint. That is, ker ⁡ ( T ) = ran ⁡ ( T ∗ ) ⊥ . {\displaystyle \ ... Define the classical differentiation operator d/dx : C1([0, 1]) → C([0, 1]) by the usual formula: ( d d x f ) ( x ) = lim h → 0 ... Let C([0, 1]) denote the space of continuous functions on the unit interval, and let C1([0, 1]) denote the space of ...

https://en.wikipedia.org/wiki/Unbounded_operator

p. C1. Bhattarai, Abha (March 17, 2014). "Business Is Brisk For Local Design Firm". The Washington Post. p. A15. Sietsma, Tom ( ... Curated signature dishes rotate daily to complement the delicious modern mainstays, which form the backbone of a menu that ...

https://en.wikipedia.org/wiki/Salamander_Washington_DC_Hotel

Bits C1, C2 and C4 use a mirrored 5-state 3-bit Gray BCD code of a Giannini Datex code type (with the first 5 states resembling ... Since the O'Brien II code forms a 9s complement, this does not give rise to particular difficulties: whenever the code word for ... information is represented as 11 binary digits in a parallel form using 11 separate lines designated D2 D4 A1 A2 A4 B1 B2 B4 C1 ... the tens represents an odd number, the code words for the decimal units are given as the 9s complements by inversion of the ...

https://en.wikipedia.org/wiki/Gillham_code

p. C1. ProQuest 123606104. Landmarks Preservation Commission Interior 1978, p. 5; National Park Service 1980, p. 2. Landmarks ... When it was completed, Town Hall's facade complemented the Georgian facade of Stephen Sondheim Theatre immediately across the ...

https://en.wikipedia.org/wiki/The_Town_Hall_(New_York_City)

Although each Cambridge English exam targets a specific level (e.g. Cambridge English C1 Advanced at Level C1) there is some ... It is designed to complement the Common European Framework of Reference for Languages (CEFR), building on Cambridge English ... For example, in C1 Advanced, a Cambridge English Scale score of 195 represents a grade B and indicates that the candidate is at ... For example: the criteria required to meet CEFR Level B2 are identical for B2 First and C1 Advanced. Although the raw marks ...

https://en.wikipedia.org/wiki/Cambridge_English_Scale

In the initial position, White's dark-square bishop is on c1; Black's is on f8. Often shortened to dark bishop or DSB. Cf. ... coordination The quality of multiple pieces working together, mutually supporting and complementing one another. corr. An ...

https://en.wikipedia.org/wiki/Glossary_of_chess

The main roads are: The C1, (Highway 1): which starts from the Distributor Las Delicias in the state capital to the western ... Lake Maracaibo and its basin are factors that complement each other to produce high rainfall. However, for the specific case of ... which is supported and complemented by the National Police and the Venezuelan National Guard. The state of Zulia occupies an ...

https://en.wikipedia.org/wiki/Zulia

To complete the isomerization, Glu357 donates its proton to C1, the C2 hydroxyl group loses its proton and the open-chain ... arthritis without reactive oxygen species and complement". The American Journal of Pathology. 183 (4): 1144-1155. doi:10.1016/j ... which deprotonates the C1 hydroxyl group. This creates an open chain aldose. Then, the substrate is rotated about the C3-C4 ...

https://en.wikipedia.org/wiki/Glucose-6-phosphate_isomerase

... the complement of the intersection is a union of two disjoint open sets. By the connectedness of the real line there must be ... c-1} , where c ∈ A {\displaystyle c\in A} and an arbitrary upper bound b 1 {\displaystyle b_{1}} of A {\displaystyle A} ). ... One can also consider the complement of each interval, written as ( − ∞ , a n ) ∪ ( b n , ∞ ) {\displaystyle (-\infty ,a_{n})\ ...

https://en.wikipedia.org/wiki/Nested_intervals

Complement Ther Med. 2018;37:64-68. doi:10.1016/j.ctim.2018.01.014 (CS1 maint: multiple names: authors list, CS1 maint: DOI ... doi:10.1097/01.phm.0000247633.68694.c1. PMID 17117004. Frieberg, AH; Vinke, TH. "Sciatica and the sacroiliac joint". Clin ... J Altern Complement Med. 2010;16(12):1285-1290. doi:10.1089/acm.2010.0031 Hoffman MD, Agnish V. Functional outcome from ...

https://en.wikipedia.org/wiki/Sacroiliac_joint_dysfunction

in Beaumont, Texas in 1944-1945, Hull was close to a type C1-M-AV1. Specs: 12 knots (22 km/h; 14 mph) max, length: 338 ft (103 ... crew complement: 64, propulsion: cross-compound turbines, single propeller. SS Matchless renamed USS Aludra in 1952 SS Flying ... Two of design types were modified type C1 ships and two were modified type C2 ships. The United Fruit Company operated many of ... Victory ships Liberty ship Type C1 ship Type C2 ship Type C3 ship United States Merchant Marine Academy List of auxiliaries of ...

https://en.wikipedia.org/wiki/Type_R_ship

Finland lost three more ships (the torpedo boats C1, C2 and C3) in supporting the British campaign in the Baltic Sea. The three ... system was taken into use in 2005 at the Uusimaa Brigade and the older truck-mounted RBS-15 missiles have been complemented ...

https://en.wikipedia.org/wiki/Finnish_Navy

... c)}(-1)^{(n+1)}}{(n+1)^{b}}}=\zeta (a,{\bar {b}},c)} ∑ n = 1 ∞ 1 ( n + 2 ) a ∑ n = 1 ∞ H ¯ n ( c ) ( n + 1 ) b = ζ ( a , b , c ... complement of { a 1 , ⋯ , a l } {\displaystyle \{a_{1},\cdots ,a_{l}\}} in { 1 , 2 , ⋯ , n } {\displaystyle \{1,2,\cdots ,n\}} ... c)}(-1)^{(n+1)}}{(n+1)^{b}}}=\zeta ({\bar {a}},{\bar {b}},{\bar {c}})} with H ¯ n ( c ) = − 1 + 1 2 c − 1 3 c + ⋯ {\ ...

https://en.wikipedia.org/wiki/Multiple_zeta_function

Let a complement ∁A be defined by a function c : [0,1] → [0,1] For all x ∈ U: μ∁A(x) = c(μA(x)) Axiom c1. Boundary condition c( ... μA(x) is defined as the degree to which x belongs to A. Let ∁A denote a fuzzy complement of A of type c. Then μ∁A(x) is the ... Let A and B be fuzzy sets that A,B ⊆ U, u is any element (e.g. value) in the U universe: u ∈ U. Standard complement μ ¬ A ( u ... Involutions c is an involution, which means that c(c(a)) = a for each a ∈ [0,1] c is a strong negator (aka fuzzy complement). A ...

https://en.wikipedia.org/wiki/Fuzzy_set_operations

Zinoman, Jason (February 18, 2012). "When Teachers Go Bad, They Get Animated". The New York Times (New York ed.). p. C1. Webb, ... he complemented the "soft, pastel-colored style that blunts the obnoxiousness of some of the jokes without blurring out the ...

https://en.wikipedia.org/wiki/China,_IL

... b0 und c1. The desire arose later to regain the old bells which had been sold off and to complement them with new, more ...

https://en.wikipedia.org/wiki/St._Patrokli,_Soest

p. C1. ProQuest 147685202. Cook, Richard A. (December 2008). "A General History of Glen Echo Park". Glen Echo - Cabin John ... and immediately set to building a stone citadel of culture to complement their real estate and resort enterprises. Opened on ...

https://en.wikipedia.org/wiki/Glen_Echo_Park_(Maryland)

Acquired C1 esterase inhibitor deficiency Acute urticaria Adrenergic urticaria Anaphylaxis Aquagenic urticaria Cholinergic ... Complement deficiency DiGeorge syndrome (DiGeorge anomaly, thymic hypoplasia) Graft-versus-host disease Griscelli syndrome ... Childhood dermatomyositis Childhood discoid lupus erythematosus Childhood systemic lupus erythematosus Complement deficiency ...

https://en.wikipedia.org/wiki/List_of_skin_conditions

SOM-C1, C2 and J variants will feature a data-link for man-in-the-loop update of a waypoint and terminal stage of the missile.[ ... It uses GPS as its primary mode of guidance complemented by an advanced Inertial Navigation System and a radar-based Terrain ... SOM-C1: inertial/GPS/imaging infrared guidance, high-explosive fragmentation warhead for use against mobile targets. Still in ...

https://en.wikipedia.org/wiki/SOM_(missile)

The complement of a knot in S3 is aspherical, by the sphere theorem Metric spaces with nonpositive curvature in the sense of ... Some references drop the requirement on c1 in their definition of "symplectically aspherical." However, it is more common for ...

https://en.wikipedia.org/wiki/Aspherical_space

Brown states the complement as 99 and Johnston states the complement of Canadian ships at 112 (7 officers and 105 ratings). Mk ... joined the Mid-Ocean Escort Force as a member of the convoy escort group C-1. Orangeville spend the rest of the Second World ... The ships had a complement of 120. The ships were powered by two Admiralty three-drum boilers which created 2,750 indicated ...

https://en.wikipedia.org/wiki/HMS_Hedingham_Castle_(K491)

An international collaborative study to establish the 1st [‎first]‎ WHO international standards for C1-inhibitor, plasma and ... Browsing by Subject "Complement C1 Inhibitor Protein". 0-9. A. B. C. D. E. F. G. H. I. J. K. L. M. N. O. P. Q. R. S. T. U. V. W ...

https://extranet.who.int/iris/restricted/browse?authority=Complement+C1+Inhibitor+Protein&type=mesh

... is not lysed by human complement. Nevertheless, HIV-1 activates complement via the classical pathway independent of antibody, ... and C3b deposition facilitates infection of complement receptor … ... HIV and complement: role of the complement system in HIV infection. Marschang P, Ebenbichler CF, Dierich MP. Marschang P, et al ... Human immunodeficiency virus type 1 activates the classical pathway of complement by direct C1 binding through specific sites ...

https://pubmed.ncbi.nlm.nih.gov/1744579

We investigated the role of complement in the pathogenesis of the demyelinating polyneuropathy that occurs in some patients ... Complement C1 / analysis * Complement C3 / analysis * Complement C5 / analysis * Complement System Proteins / immunology* ... Complement-mediated demyelination in patients with IgM monoclonal gammopathy and polyneuropathy N Engl J Med. 1990 Mar 8;322(10 ... In addition, terminal-complement complex that was not associated with S protein was detected in myelin sheaths. It appeared ...

https://pubmed.ncbi.nlm.nih.gov/1689461/?dopt=Abstract

Insights into IgM-mediated complement activation based on in situ structures of IgM-C1-C4b.. Sharp, Thomas H; Boyle, Aimee L; ... The IgM-C1-C4b complexes revealed C4b product release as the temperature-limiting step in complement activation. Both IgM ... C1 binds IgM through widely spread C1q-collagen helices, with C1r proteases pointing outward and C1s bending downward and ... We present cryoelectron tomography structures of IgM, C1, and C4b complexes formed on antigen-bearing lipid membranes by normal ...

https://pesquisa.bvsalud.org/saudepublica/resource/pt/mdl-31147461

Start Over You searched for: Subjects Complement C1 Inhibitor Protein -- economics ✖Remove constraint Subjects: Complement C1 ... Complement C1 Inhibitor Protein -- adverse effects. Complement C1 Inhibitor Protein -- economics. Cost-Benefit Analysis. ... Complement C1 Inhibitor Protein -- therapeutic use. Kallikreins -- antagonists & inhibitors. Antibodies, Monoclonal, Humanized ... 1. Prophylaxis for hereditary angioedema with lanadelumab and C1 Inhibitors: effectiveness and value : final evidence report ...

https://collections.nlm.nih.gov/?f%5Bdrep2.subjectAggregate%5D%5B%5D=Complement+C1+Inhibitor+Protein+--+economics

Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... Biological: C1-esterase inhibitor. Experimental: C1-INH - medium-volume dose A medium-volume dose of C1-INH will be ... Experimental: C1-INH - low-volume dose A low-volume dose of C1-INH will be administered subcutaneously twice a week for up to ... The aim of this study is to assess the long-term safety of C1-esterase inhibitor (C1-INH) in preventing hereditary angioedema ( ...

https://clinicaltrials.gov/ct2/show/NCT02316353

Complement C3 is a blood test that measures the activity of a certain protein. ... There are nine major complement proteins. They are labeled C1 through C9. This article describes the test that measures C3. ... The complement cascade is a series of reactions that take place in the blood. The cascade activates the complement proteins. ... When the complement system is turned on during inflammation, levels of complement proteins may go down. For example, people ...

https://medlineplus.gov/ency/article/003539.htm

C1-Inhibitor deficiency and angioedema. In: K. Whaley, ed. Complement in Health and Disease. MTP Press Limited; 1987;p 53. ... Question 1. What is the difference between the C1 Inhibitor, Protein test and the C1 Inhibitor, Functional test? The C1 ... The C1 Inhibitor, Functional test (test code 297) measures the relative functionality of an individuals C1 esterase inhibitor ... Concentrations of the C1 inhibitor protein are normal (11-26 ng/mL), but the protein is dysfunctional (functional activity ,40 ...

https://www.questdiagnostics.com/healthcare-professionals/clinical-education-center/faq/faq54

Chromosome: 3; NC_000003.12 (52451100..52454041, complement). Total number of exons:. 6. ... TNNC1 troponin C1, slow skeletal and cardiac type. Gene ID: 7134, updated on 7-Sep-2023. Gene type: protein coding. Also known ...

https://www.ncbi.nlm.nih.gov/gtr/genes/7134

2s Complement Division C++ Program. 2s Complement Division C++ Program. Ajitabh Gupta ... C1 UNIT 8 Vocabulary revision. C1 UNIT 8 Vocabulary revision. Ольга Мухина ...

https://www.scribd.com/document/50402296/Siblings-Other-Torture-Devices-Excerpt-2-A-Novel-by-Kenya-D-Williamson

Bidirectionalization transformation based on automatic derivation of view complement functions. ICFP 2007: 47-58. ...

https://dblp.uni-trier.de/pid/28/6926.html

The complement system plays an important part in defense against pyogenic organisms. ... The complement system is part of the innate immune system. ... Deficiencies of early classical pathway components (C1, C4, C2 ... Cases of complement deficiency have helped defined the role of complement in host defense. [4] A registry of complement ... The complement system is part of the innate immune system. The complement system plays an important part in defense against ...

http://emedicine.medscape.com/article/886128-overview

"Borrelia burgdorferi BBK32 Inhibits the Classical Pathway by Blocking Activation of the C1 Complement Complex", PLoS Pathogens ... As a result, C1 is trapped in an inactive form.[33] Genome[edit]. B. burgdorferi (B31 strain) was the third microbial genome ... "Human complement control and complement evasion by pathogenic microbes - Tipping the balance". Molecular Immunology. 56 (3): ... This is part of a complement system evasion strategy that leads to downstream blocking of immune response.[32] ...

https://en.wikipedia.org/wiki/Borrelia_burgdorferi

MeSH Terms: Adult; Case-Control Studies; Complement C1/urine; Cystine/analysis; Cystinuria/urine*; Down-Regulation; Endosomal ...

https://tools.niehs.nih.gov/portfolio/index.cfm?do=portfolio.publicationDetail&id=3550810

Complement C1 Inhibitor Protein D12.776.872.140.500. Complement Fixation Tests E5.478.594.760.150. Complement Hemolytic ... Receptors, Complement 3b D23.50.301.264.35.600 D23.50.301.264.35.608. D23.101.100.110.600 D23.101.100.110.608. Receptors, CXCR4 ...

https://decs.bvs.br/P/mnchg2008.htm

6. Complement C1-inhibitor expression in Alzheimers disease.. Veerhuis R; Janssen I; Hoozemans JJ; De Groot CJ; Hack CE; ... 8. Regulation of the synthesis of C1 subcomponents and C1-inhibitor.. Gulati P; Lemercier C; Guc D; Lappin D; Whaley K. Behring ... Antibody-independent activation of C1. I. Differences in the mechanism of C1 activation by nonimmune activators and by immune ... Synthesis of complement proteins in the human chorion is differentially regulated by cytokines.. Goldberg M; Luknar-Gabor N; ...

https://pubmedhh.nlm.nih.gov/biomarkers/search.php?id=10630213&mod=related&page=1&outid=&proj=

Identification of the C1q-binding sites of human C1r and C1s: a refined three-dimensional model of the C1 complex of complement ... Cooper, N. R. The classical complement pathway: activation and regulation of the first complement component. Adv. Immunol. 37, ... Structural biology of C1: dissection of a complex molecular machinery. Immunol. Rev. 180, 136-145 (2001). ... Arlaud, G. J., Colomb, M. G. & Gagnon, J. A functional model of the human C1 complex: emergence of a functional model. Immunol ...

https://www.nature.com/articles/s41572-020-0194-9?error=cookies_not_supported&code=3beb1884-a652-4ce1-b49d-d1d910b0b96f

looking for Full complement cylindrical roller bearings NCF2876V ? Pioneer Machinery Source Our expert FULL ROW OF CYLINDRICAL ... Full complement cylindrical roller bearings NJG2326VH. C1 max.:28 mm; Specific dynamic load factor K:100 N/mm²; B:32 mm; G:M ... Full complement cylindrical roller bearings NCF2876V SPECIFICATIONS. *. Cant find what youre 28 mm C1 max. looking for Full ... Customers Who Viewed Full complement cylindrical roller bearings NCF2876V Also Viewed. *. Full complement cylindrical roller ...

https://www.ga-source.com/kathmandu-15804816-full-complement-cylindrical-roller-bearings-ncf2876v.html

The primary function of C1 inhibitor is to regulate the activation of the complement and intrinsic coagulation (contact system ... HUMAN C1-ESTERASE INHIBITOR (UNII: 6KIC4BB60G) (HUMAN C1-ESTERASE INHIBITOR - UNII:6KIC4BB60G) HUMAN C1-ESTERASE INHIBITOR. 500 ... HUMAN C1-ESTERASE INHIBITOR (UNII: 6KIC4BB60G) (HUMAN C1-ESTERASE INHIBITOR - UNII:6KIC4BB60G) HUMAN C1-ESTERASE INHIBITOR. 500 ... CINRYZE- human c1-esterase inhibitor injection, powder, lyophilized, for solution. CINRYZE- human c1-esterase inhibitor kit. If ...

https://dailymed.nlm.nih.gov/dailymed/lookup.cfm?setid=d53a911f-070d-498a-8a61-baab72b9d0fe&version=39

The system consists of nine complement component proteins, called C1, C2, C3, and so on up to C9. When activated, these ... Complement factors H and I (CFH and CFI) are proteins that inhibit the complement system and keep it from attacking the bodys ... The complement system is a large group of proteins that work together to trigger inflammatory and immune responses that defend ... "Genetic studies to date have shown that the complement system plays an important role in AMD. These new studies enhance our ...

https://www.nei.nih.gov/about/news-and-events/news/three-studies-point-same-risk-gene-age-related-macular-degeneration

The large UFO-shaped objects are low density lipoprotein and the six-armed protein is complement C1. The red blood cell is ...

https://www.asbmb.org/meetings-events/pdb50

Includes B- (humoral) or T-lymphocyte deficiency, complement deficiencies (particularly C1, C2, C3, and C4 deficiencies), and ...

https://www.medscape.com/viewarticle/923407_slide

4. Mutants of complement component C3 cleaved by the C4-specific C1-s protease.. Mathias P; Carrillo CJ; Zepf NE; Cooper NR; ... Effect of plicatic acid on human serum complement includes interference with C1 inhibitor function.. Giclas PC. J Immunol; 1982 ... Complement C2 receptor inhibitor trispanning and the beta-chain of C4 share a binding site for complement C2.. Inal JM; ... Functional role of the linker between the complement control protein modules of complement protease C1s.. Bally I; Rossi V; ...

https://pubmedhh.nlm.nih.gov/biomarkers/search.php?id=7650393&mod=related&page=1&outid=&proj=

Increases serum levels of complement 1 (C1) esterase inhibitor, which results in increased serum levels of complement C4 in ...

https://www.drugs.com/monograph/danazol.html

J Altern Complement Med. 2004;10(6):959-65. DOI:10.1089/acm.2004.10.959 e 54,01010 Lee MK, Chang SB, Lee HS, Kim HS. Effects of ... DOI:10.1097/01.JNR.0000347644.35251.c1 e ponto Zhiyin(B67).55 Chung U, Hung L, Nancy O, Kuo S. Effects of LI4 and BL 67 ... J Altern Complement Med. 2004;10(6):959-65. DOI:10.1089/acm.2004.10.959,1414 Kashanian M, Shahali S. Effects of acupressure at ... J Altern Complement Med. 2004;10(6):959-65. DOI:10.1089/acm.2004.10.959,1414 Kashanian M, Shahali S. Effects of acupressure at ...

https://scielosp.org/article/rsp/2015.v49/9/pt/

Both C3 and C5 of the complement system can become activated through FIXa, FXa, FXIa, thrombin, and plasmin, and C1 can be ... Furthermore, the coagulation and complement systems are closely intertwined. It has been shown that the complement system, and ... In turn, the complement system can contribute to a procoagulatory response by activating thrombin via components of the lectin ... Targeted donor complement blockade after brain death prevents delayed graft function in a nonhuman primate model of kidney ...

https://journals.lww.com/transplantjournal/Fulltext/2023/06000/Pathophysiological_Changes_in_the_Hemostatic.13.aspx

The complement system is a group of nearly 60 proteins that are in blood plasma or on the surface of some cells. (medlineplus.gov)
Rarely, people may inherit deficiency of some complement proteins. (medlineplus.gov)
There are nine major complement proteins. (medlineplus.gov)
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)
Genes that encode the proteins of complement components or their isotypes are distributed throughout different chromosomes, with 19 genes comprising 3 significant complement gene clusters in the human genome. (medscape.com)
The important components of this system are various cell membrane-associated proteins such as complement receptor 1 (CR1), complement receptor 2 (CR2), and decay accelerating factor (DAF). (medscape.com)
1. Cytokines associated with amyloid plaques in Alzheimer's disease brain stimulate human glial and neuronal cell cultures to secrete early complement proteins, but not C1-inhibitor. (nih.gov)
19. Synthesis of complement proteins in the human chorion is differentially regulated by cytokines. (nih.gov)
The new research, which was funded in part by the National Institutes of Health, solidifies a link between AMD and genes encoding the complement system, a set of proteins that plays a central part in immune responses and inflammation. (nih.gov)
The complement system is a large group of proteins that work together to trigger inflammatory and immune responses that defend our bodies against infection. (nih.gov)
The system consists of nine complement component proteins, called C1, C2, C3, and so on up to C9. (nih.gov)
Complement factors H and I (CFH and CFI) are proteins that inhibit the complement system and keep it from attacking the body's own cells. (nih.gov)
The C1 esterase inhibitor protein is required to control the 'complement' and 'contact' systems, collections of proteins in the blood that fight against infection and cause inflammation. (europa.eu)
For example, in the complement proteases, the CUB domains mediate dimerisation and binding to collagen-like regions of target proteins (e.g. (embl.de)
Sequence analysis has revealed the presence of 31 copies of an extracellular domain, here called CUB, in 16 functionally diverse proteins such as the dorso-ventral patterning protein tolloid, bone morphogenetic protein 1, a family of spermadhesins, complement subcomponents Cls/Clr and the neuronal recognition molecule A5. (embl.de)
Description: The complement component proteins, C2, C3, C4 and C5, are potent anaphylatoxins that are released during complement activation. (wlsolutions.be)

The aim of this study is to assess the long-term safety of C1-esterase inhibitor (C1-INH) in preventing hereditary angioedema (HAE) attacks when it is administered under the skin of subjects with HAE. (clinicaltrials.gov)
CINRYZE is a C1 esterase inhibitor indicated for routine prophylaxis against angioedema attacks in adults, adolescents, and pediatric patients (6 years of age and older) with Hereditary Angioedema (HAE). (nih.gov)
Serious arterial and venous thromboembolic (TE) events have been reported at the recommended dose of C1 Esterase Inhibitor (Human) products, including CINRYZE, following administration in patients with HAE. (nih.gov)
TE events have been reported following administration of a C1 Esterase Inhibitor (Human) product when used off-label at higher than labeled doses. (nih.gov)
However, fresh frozen plasma (FFP), antifibrinolytics, C1 esterase inhibitor (C1-INH), ecallantide, and icatibant can be used to manage bradykinin-mediated angioedema. (medscape.com)
The active substance in Ruconest, conestat alfa, is a copy of the C1 esterase inhibitor protein and works in the same way as the natural human protein. (europa.eu)
Ruconest was studied in two main studies involving a total of 70 adults and adolescents with hereditary angioedema caused by low levels of C1 esterase inhibitor protein. (europa.eu)

10. C1 subcomponent complexes: basic and clinical aspects. (nih.gov)
5. Evidence for the involvement of arginine 462 and the flanking sequence of human C4 beta-chain in mediating C5 binding to the C4b subcomponent of the classical complement pathway C5 convertase. (nih.gov)
20. Functional analysis of activated C1s, a subcomponent of the first component of human complement, by monoclonal antibodies. (nih.gov)
From NCBI Gene: This gene encodes a serine protease, which is a major constituent of the human complement subcomponent C1. (nih.gov)

Nevertheless, HIV-1 activates complement via the classical pathway independent of antibody, and C3b deposition facilitates infection of complement receptor-bearing cells. (nih.gov)
The complement cascade consists of 3 separate pathways that converge in a final common pathway. (medscape.com)
Lectins activate the lectin pathway in a manner similar to the antibody interaction with complement in the classical pathway. (medscape.com)
Evidence for two classes of nonimmune activators of the classical pathway of complement. (nih.gov)
9. Synthesis of classical pathway complement components by chondrocytes. (nih.gov)
7. A single arginine to tryptophan interchange at beta-chain residue 458 of human complement component C4 accounts for the defect in classical pathway C5 convertase activity of allotype C4A6. (nih.gov)
11. Distal recognition site for classical pathway convertase located in the C345C/netrin module of complement component C5. (nih.gov)
The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY . (nih.gov)
The multiprotein complex C1 initiates the classical pathway of complement activation on binding to antibody-antigen complexes, pathogen surfaces, apoptotic cells, and polyanionic structures. (rcsb.org)
Mammalian complement subcomponents C1s/C1r, which form the calcium-dependent complex C1, the first component of the classical pathway of the complement system. (embl.de)

We investigated the role of complement in the pathogenesis of the demyelinating polyneuropathy that occurs in some patients with IgM monoclonal gammopathy. (nih.gov)
Cases of complement deficiency have helped defined the role of complement in host defense. (medscape.com)

8. Regulation of the synthesis of C1 subcomponents and C1-inhibitor. (nih.gov)

Insights into IgM-mediated complement activation based on in situ structures of IgM-C1-C4b. (bvsalud.org)
We present cryoelectron tomography structures of IgM , C1, and C4b complexes formed on antigen -bearing lipid membranes by normal human serum at 4 °C. The IgM -C1-C4b complexes revealed C4b product release as the temperature -limiting step in complement activation . (bvsalud.org)
Based on these data, we present mechanistic models for antibody-mediated, C1q-transmitted activation of C1 and for C4b deposition, while further conformational rearrangements are required to form C3 convertases. (bvsalud.org)
at the same time, the inflammation promoted by complement activation can result in cellular damage when not kept in check. (medscape.com)
4. Antibody-independent activation of C1. (nih.gov)
I. Differences in the mechanism of C1 activation by nonimmune activators and by immune complexes: C1r-independent activation of C1s by cardiolipin vesicles. (nih.gov)
Activation of kinin-kallikrein and complement pathways by oversulfated-chondroitin-sulfate (OSCS) has been linked with recent heparin-associated adverse clinical events. (nih.gov)
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 . (nih.gov)
Considerable flexibility in C1s likely facilitates C1 complex formation, activation of C1s by C1r, and binding and activation of downstream substrates C4 and C4b-bound C2 to initiate the reaction cascade. (rcsb.org)
The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. (antibodies-online.com)
Three major pathways of complement activation have been described that are triggered independently, namely the classical, the lectin, and the alternative pathways, all of which merge at the step of C3 activation. (nih.gov)
Immune sensing of pathogens or foreign objects can induce local and systemic complement activation and subsequent inflammation at early timepoints during infection. (nih.gov)
however, over-activation or dysregulation of the complement cascade can cause collateral damage of cells and tissues. (nih.gov)
For example, complement dysregulation has been observed in severe COVID-19 disease where over-activation of the complement system induces endothelial cell injury and death, leading to initiation of clotting cascades. (nih.gov)

Direct and indirect immunofluorescence and immunoperoxidase assays showed colocalization along the myelin sheaths of peripheral-nerve fibers of monoclonal protein with complement components C1q, C3d, and C5. (nih.gov)
In addition, terminal-complement complex that was not associated with S protein was detected in myelin sheaths. (nih.gov)
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)
The large UFO-shaped objects are low density lipoprotein and the six-armed protein is complement C1. (asbmb.org)
3. Active sites in complement components C5 and C3 identified by proximity to indels in the C3/4/5 protein family. (nih.gov)
13. Functional role of the linker between the complement control protein modules of complement protease C1s. (nih.gov)
15. Murine complement component C4 and sex-limited protein: identification of amino acid residues essential for C4 function. (nih.gov)

17. Complement C2 receptor inhibitor trispanning and the beta-chain of C4 share a binding site for complement C2. (nih.gov)
C3d is a terminal degradation product of C3 that plays an important role in modulation of the adaptive immune response through the interaction with complement receptor type 2 (CR2). (wlsolutions.be)

How antigen -bound IgM activates complement - immune responses remains unclear. (bvsalud.org)

In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). (medscape.com)
The complement system is an evolutionarily conserved component of innate immunity that serves as a first line of defense against infection. (nih.gov)

Complement pathways and deficiencies. (medscape.com)
There are additional reports of interaction between individual complement components and innate signaling pathways, including Toll-Like Receptors. (nih.gov)
The mechanisms by which complement components, receptors, and pathways contribute to protective immune responses or pathogenesis remain to be fully elucidated. (nih.gov)

A North African study of molecular basis of complement factor I deficiency in atypical hemolytic and uremic syndrome patients suggested that the Ile357Met mutation may be a founding effect. (medscape.com)
8. Molecular basis of complement resistance of human melanoma cells expressing the C3-cleaving membrane protease p65. (nih.gov)

12. The structure and function of the first component of complement: genetic engineering approach (a review). (nih.gov)
1. Complement component C5: engineering of a mutant that is specifically cleaved by the C4-specific C1s protease. (nih.gov)
4. Mutants of complement component C3 cleaved by the C4-specific C1-s protease. (nih.gov)
10. Identification of a catalytic exosite for complement component C4 on the serine protease domain of C1s. (nih.gov)
12. Structural homologies of component C5 of human complement with components C3 and C4 by neutron scattering. (nih.gov)
18. Third component of trout complement. (nih.gov)
C1s associates with two other complement components C1r and C1q in order to yield the first component of the serum complement system. (nih.gov)
Mammalian complement-activating component of Ra-reactive factor (RARF), a protease that cleaves the C4 component of complement. (embl.de)

14. Effect of plicatic acid on human serum complement includes interference with C1 inhibitor function. (nih.gov)

Here we have determined the structure of a complex between the CUB1-EGF-CUB2 fragments of C1r and C1s to reveal the C1r-C1s interaction that forms the core of C1. (rcsb.org)

The complement system is part of the innate immune system. (medscape.com)
Although the complement system is part of the body's innate, relatively nonspecific defense against pathogens, its role is hardly primitive or easily understood. (medscape.com)

C1 binds IgM through widely spread C1q- collagen helices, with C1r proteases pointing outward and C1s bending downward and interacting with surface-attached C4b, which further interacts with the adjacent IgM -Fab2 and globular C1q-recognition unit. (bvsalud.org)

The complement cascade is a series of reactions that take place in the blood. (medlineplus.gov)
Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. (medscape.com)
New studies point to the complex interplay between the complement cascade and adaptive immune response, and complement is also being studied in association with ischemic injury as a target of therapy. (medscape.com)
Complement-associated molecules, known as C1 through C9, operate in a cascade of enzymatic reactions that can ultimately lead to lysis of infected cells and opsonization of pathogens to aid in phagocytic clearance. (nih.gov)

Virions activated the C1 complex, reconstituted from C1q, proenzyme C1r, and 125I-labeled proenzyme C1s, to an extent comparable with that obtained with immunoglobulin G-ovalbumin immune complexes. (nih.gov)
In the fluid phase, sgp41 activated the C1 complex in a dose- and time-dependent manner, more efficiently than aggregated Ig, but less efficiently than immune complexes. (nih.gov)

Bean KV, Massey HD, Gupta G. Mediators of inflammation: complement. (medlineplus.gov)

C1- inhibitor (C1-INH) and complement assay levels and function are normal. (medscape.com)

Complement deficiencies are said to comprise between 1 and 10% of all primary immunodeficiencies. (medscape.com)
[ 4 ] A registry of complement deficiencies has been established as a means to promote joint projects on treatment and prevention of diseases associated with defective complement function. (medscape.com)
This article outlines some of the disease states associated with complement deficiencies and their clinical implications. (medscape.com)
Deficiencies in complement predispose patients to infection via 2 mechanisms: (1) ineffective opsonization and (2) defects in lytic activity (defects in MAC). (medscape.com)
Specific complement deficiencies are also associated with an increased risk of developing autoimmune disease, such as SLE. (medscape.com)

Human immunodeficiency virus type 1 (HIV-1), in contrast to animal retroviruses such as murine leukemia virus, is not lysed by human complement. (nih.gov)

We conclude that demyelination in polyneuropathy associated with IgM monoclonal gammopathy may be mediated by complement. (nih.gov)

A low-volume dose of C1-INH will be administered subcutaneously twice a week for up to 52 weeks (up to 146 weeks extension period). (clinicaltrials.gov)

In addition to functioning in the above-mentioned enzymatic cascades, individual complement components have been shown to play other critical roles in immune responses. (nih.gov)
The main objective of this program is to support studies that accelerate our understanding of the roles of complement components and/or receptors in the initiation, magnitude, maintenance, and quality of immune responses involved in pathogenic infections, vaccination, post-infection sequelae, autoimmunity, allergy, or transplantation. (nih.gov)
The work to be encouraged includes studies of the roles of complement components (molecules and/or receptors) during immune responses. (nih.gov)
Development of in vivo animal models or cell/tissue-specific in vitro systems for studying the roles of complement in regulating immune responses. (nih.gov)

Each eligible subject will enter the treatment phase, wherein subjects will be randomized to treatment with either low- or medium-volume C1-INH. (clinicaltrials.gov)

Complement your expertise with clinical consultations from Quest. (questdiagnostics.com)
Some new clinical entities are linked with partial complement defects. (medscape.com)

It appeared that alterations in myelin geometry caused by the separation of myelin lamellae corresponded to sites at which terminal-complement complex was deposited. (nih.gov)
This complex process enables the C1 to simulate the experience of playing either a German grand or a Japanese grand, and were chosen to reflect a broad musical spectrum providing faithful recreations of dark, subtle and intense classical music through to bright and powerful jazz and pop music. (korg.com)

Genetic studies to date have shown that the complement system plays an important role in AMD. (nih.gov)

C3 and C4 are the most commonly measured complement components. (medlineplus.gov)
13. Early complement components in Alzheimer's disease brains. (nih.gov)
Complement components can also be produced in a broad range of immune and other cell types, such as endothelial cells. (nih.gov)
The results of such studies will inform the development of vaccines or therapeutics that target complement components. (nih.gov)

3. Synthesis and regulation of C1 inhibitor in human skin fibroblasts. (nih.gov)

Complement system in disease. (medlineplus.gov)
The complement system. (medlineplus.gov)
The complement system plays an important part in defense against pyogenic organisms. (medscape.com)
These findings underscore the duality of the complement system. (medscape.com)
Knowledge about the complement system is expanding. (medscape.com)
An intricate system regulates complement activity. (medscape.com)
The research suggests that drugs designed to suppress the complement system, and particularly C3, could be useful against AMD. (nih.gov)

With KORG's unique history of musical instrument design and audio technology, the C1 offers levels of playability and versatility that will exceed the expectations of the most demanding pianist. (korg.com)

A complement test may be used to monitor people with an autoimmune disorder . (medlineplus.gov)

Anatomy15

Organisms10

Diseases16

Chemicals and Drugs101

Analytical, Diagnostic and Therapeutic Techniques and Equipment25

Phenomena and Processes48

Disciplines and Occupations2

Information Science3

Health Care1

Immune complexes and complement hypercatabolism in patients with leprosy. (1/321)

Binding of soluble immune complexes to Raji lymphocytes. Role of receptors for complement components, C1q and C3-C3b. (2/321)

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)

Isolation of two forms of activated C1s, a subcomponent of the first component of rabbit complement. (4/321)

Complement C1s activation in degenerating articular cartilage of rheumatoid arthritis patients: immunohistochemical studies with an active form specific antibody. (5/321)

Possible mechanisms of the first step of the classical complement activation pathway: binding and activation of C1. (6/321)

C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. (7/321)

Inhibitory effect of bilirubin on complement-mediated hemolysis. (8/321)

Complement C3

Complement C4

Complement C4a

Complement C3a

Complement C1q

Complement C5a

Complement Activation

Complement C4b

Complement C5

Complement C3b

Complement System Proteins

Complement C6

Complement C3c

Complement C3d

Complement C2

Complement C9

Receptors, Complement

Complement C1s

Complement Membrane Attack Complex

Complement C1r

Complement Inactivator Proteins

Complement C7

Complement C3-C5 Convertases

Complement Factor B

Complement Pathway, Alternative

Complement Pathway, Classical

Complement C8

Complement C1

Receptors, Complement 3b

Complement Factor H

Complement C5b

Complement C2a

Receptor, Anaphylatoxin C5a

Complement Activating Enzymes

Complement Inactivating Agents

Complement Hemolytic Activity Assay

Complement C1 Inactivator Proteins

Receptors, Complement 3d

Anaphylatoxins

Complement Fixation Tests

Complement Factor D

Complement Factor I

Complement C4b-Binding Protein

Complement C3b Inactivator Proteins

Antigens, CD55

Complement C3-C5 Convertases, Classical Pathway

Complement C2b

Antigens, CD59

Cobra Venoms

Antigen-Antibody Complex

Steroid 21-Hydroxylase

Complement C3-C5 Convertases, Alternative Pathway

Complement C1 Inhibitor Protein

Immunoglobulin G

Hemolysis

Complement C3 Convertase, Alternative Pathway

Complement C5 Convertase, Classical Pathway

Molecular Sequence Data

Complement C3 Convertase, Classical Pathway

Antigens, CD46

Opsonin Proteins

Blood Proteins

Lupus Erythematosus, Systemic

Complement C5 Convertase, Alternative Pathway

Phagocytosis

Amino Acid Sequence

Complement Pathway, Mannose-Binding Lectin

Properdin

Complement C5a, des-Arginine

Mice, Inbred C57BL

Macrophage-1 Antigen

Protein Binding

Neutrophils

Base Sequence

Kidney Glomerulus

Serum

Glomerulonephritis, Membranoproliferative

Immunoglobulin M

Schistosoma

Genetic Complementation Test