Complement C3: A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.Complement C1 Inactivator Proteins: Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.Complement C3b Inactivator Proteins: Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.Complement C4: 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.Complement Inactivator Proteins: Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.Complement C4a: 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.Complement C3a: The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.Complement Activation: 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.Complement C1q: A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.Complement C5: C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.Complement C5a: The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.Complement C4b: 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).Complement C3b: The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.Complement System Proteins: Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).Complement C2: A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Complement C6: A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.Complement C3c: 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.Complement C3d: A 302-amino-acid fragment in the alpha chain (672-1663) of C3b. It is generated when C3b is inactivated (iC3b) and its alpha chain is cleaved by COMPLEMENT FACTOR I into C3c, and C3dg (955-1303) in the presence COMPLEMENT FACTOR H. Serum proteases further degrade C3dg into C3d (1002-1303) and C3g (955-1001).Complement C9: A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.Receptors, Complement: Molecules on the surface of some B-lymphocytes and macrophages, that recognize and combine with the C3b, C3d, C1q, and C4b components of complement.Complement C1s: A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).Complement C3-C5 Convertases: Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.Complement Membrane Attack Complex: A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.Complement C1r: A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.Complement Factor B: 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 Pathway, Alternative: Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Complement C7: A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.Complement Pathway, Classical: Complement activation initiated by the binding of COMPLEMENT C1 to ANTIGEN-ANTIBODY COMPLEXES at the COMPLEMENT C1Q subunit. This leads to the sequential activation of COMPLEMENT C1R and COMPLEMENT C1S subunits. Activated C1s cleaves COMPLEMENT C4 and COMPLEMENT C2 forming the membrane-bound classical C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Complement C8: A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.Complement C1: The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.Complement Factor H: An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).Anaphylatoxins: Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.Complement Activating Enzymes: 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.Properdin: A 53-kDa protein that is a positive regulator of the alternate pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It stabilizes the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb) and protects it from rapid inactivation, thus facilitating the cascade of COMPLEMENT ACTIVATION and the formation of MEMBRANE ATTACK COMPLEX. Individuals with mutation in the PFC gene exhibit properdin deficiency and have a high susceptibility to infections.Receptors, Complement 3b: Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.Complement C5b: The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.Complement C2a: 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).Receptor, Anaphylatoxin C5a: A G-protein-coupled receptor that signals an increase in intracellular calcium in response to the potent ANAPHYLATOXIN peptide COMPLEMENT C5A.Complement Inactivating Agents: Compounds that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host.Complement Hemolytic Activity Assay: A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.Receptors, Complement 3d: Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.Hemolysis: The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.Complement Fixation Tests: Serologic tests based on inactivation of complement by the antigen-antibody complex (stage 1). Binding of free complement can be visualized by addition of a second antigen-antibody system such as red cells and appropriate red cell antibody (hemolysin) requiring complement for its completion (stage 2). Failure of the red cells to lyse indicates that a specific antigen-antibody reaction has taken place in stage 1. If red cells lyse, free complement is present indicating no antigen-antibody reaction occurred in stage 1.Complement Factor D: 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.Antigen-Antibody Complex: 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.Complement Factor I: A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.Complement C4b-Binding Protein: 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).Antigens, CD55: 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.Complement C3-C5 Convertases, Classical Pathway: Important enzymes in the CLASSICAL COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.Complement C2b: The N-terminal fragment of COMPLEMENT 2, released by the action of activated COMPLEMENT C1S.Blood Proteins: Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.Antigens, CD59: 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)Lysine Carboxypeptidase: A metallocarboxypeptidase that removes C-terminal basic amino acid from peptides and proteins, with preference shown for lysine over arginine. It is a plasma zinc enzyme that inactivates bradykinin and anaphylatoxins.Beta-Globulins: Serum proteins with an electrophoretic mobility that falls between ALPHA-GLOBULINS and GAMMA-GLOBULINS.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Kinetics: The rate dynamics in chemical or physical systems.Immunoelectrophoresis: A technique that combines protein electrophoresis and double immunodiffusion. In this procedure proteins are first separated by gel electrophoresis (usually agarose), then made visible by immunodiffusion of specific antibodies. A distinct elliptical precipitin arc results for each protein detectable by the antisera.Cytochrome P-450 CYP2B1: A major cytochrome P-450 enzyme which is inducible by PHENOBARBITAL in both the LIVER and SMALL INTESTINE. It is active in the metabolism of compounds like pentoxyresorufin, TESTOSTERONE, and ANDROSTENEDIONE. This enzyme, encoded by CYP2B1 gene, also mediates the activation of CYCLOPHOSPHAMIDE and IFOSFAMIDE to MUTAGENS.Phagocytosis: The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).Cobra Venoms: 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.Steroid 21-Hydroxylase: An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).Chloromercurinitrophenols: Mercuriphenols substituted with one or more chlorine atoms and one or more nitro groups. Some of these are sulfhydryl reagents which act as chromophoric probes in enzymes and other proteins.Complement C3-C5 Convertases, Alternative Pathway: Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.Amino Acid Sequence: 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.Angioedema: Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.Complement C1 Inhibitor Protein: An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Complement C3 Convertase, Alternative Pathway: 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.Kaolin: The most common mineral of a group of hydrated aluminum silicates, approximately H2Al2Si2O8-H2O. It is prepared for pharmaceutical and medicinal purposes by levigating with water to remove sand, etc. (From Merck Index, 11th ed) The name is derived from Kao-ling (Chinese: "high ridge"), the original site. (From Grant & Hackh's Chemical Dictionary, 5th ed)Complement C5 Convertase, Classical 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.Serum Globulins: All blood proteins except albumin ( = SERUM ALBUMIN, which is not a globulin) and FIBRINOGEN (which is not in the serum). The serum globulins are subdivided into ALPHA-GLOBULINS; BETA-GLOBULINS; and GAMMA-GLOBULINS on the basis of their electrophoretic mobilities. (From Dorland, 28th ed)Neutrophils: 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.Complement C3 Convertase, Classical Pathway: 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).Antigens, CD46: 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.Protein Binding: 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.Enzyme Inhibitors: Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.Opsonin Proteins: 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.Binding Sites: The parts of a macromolecule that directly participate in its specific combination with another molecule.Lupus Erythematosus, Systemic: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.Complement C5 Convertase, Alternative Pathway: 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.Hydroxybenzoate Ethers: Benzoate derivatives that contain one or more alkyl or aryl groups linked to the benzene ring structure by OXYGEN.Dihydrouracil Dehydrogenase (NADP): An oxidoreductase involved in pyrimidine base degradation. It catalyzes the catabolism of THYMINE; URACIL and the chemotherapeutic drug, 5-FLUOROURACIL.Alkynes: Hydrocarbons with at least one triple bond in the linear portion, of the general formula Cn-H2n-2.Kallikreins: Proteolytic enzymes from the serine endopeptidase family found in normal blood and urine. Specifically, Kallikreins are potent vasodilators and hypotensives and increase vascular permeability and affect smooth muscle. They act as infertility agents in men. Three forms are recognized, PLASMA KALLIKREIN (EC 3.4.21.34), TISSUE KALLIKREIN (EC 3.4.21.35), and PROSTATE-SPECIFIC ANTIGEN (EC 3.4.21.77).Electrophoresis, Polyacrylamide Gel: Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.Complement Pathway, Mannose-Binding Lectin: Complement activation triggered by the interaction of microbial POLYSACCHARIDES with serum MANNOSE-BINDING LECTIN resulting in the activation of MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. As in the classical pathway, MASPs cleave COMPLEMENT C4 and COMPLEMENT C2 to form C3 CONVERTASE (C4B2A) and the subsequent C5 CONVERTASE (C4B2A3B) leading to cleavage of COMPLEMENT C5 and assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Blood Protein Electrophoresis: Electrophoresis applied to BLOOD PROTEINS.EsterasesCarboxypeptidases A: Carboxypeptidases that are primarily found the DIGESTIVE SYSTEM that catalyze the release of C-terminal amino acids. Carboxypeptidases A have little or no activity for hydrolysis of C-terminal ASPARTIC ACID; GLUTAMIC ACID; ARGININE; LYSINE; or PROLINE. This enzyme requires ZINC as a cofactor and was formerly listed as EC 3.4.2.1 and EC 3.4.12.2.Chromatography, Gel: Chromatography on non-ionic gels without regard to the mechanism of solute discrimination.Rabbits: 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.4-Aminobutyrate Transaminase: An enzyme that converts brain gamma-aminobutyric acid (GAMMA-AMINOBUTYRIC ACID) into succinate semialdehyde, which can be converted to succinic acid and enter the citric acid cycle. It also acts on beta-alanine. EC 2.6.1.19.Complement C5a, des-Arginine: 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).Ticrynafen: A novel diuretic with uricosuric action. It has been proposed as an antihypertensive agent.UracilMice, Inbred C57BLPeptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Macrophage-1 Antigen: 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.Cells, Cultured: 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.Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Glycoproteins: Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.Recombinant Proteins: Proteins prepared by recombinant DNA technology.Oxidoreductases, N-DemethylatingBenzphetamine: A sympathomimetic agent with properties similar to DEXTROAMPHETAMINE. It is used in the treatment of obesity. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1222)Trypsin Inhibitors: Serine proteinase inhibitors which inhibit trypsin. They may be endogenous or exogenous compounds.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Neuraminic AcidsKidney Glomerulus: A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.Aryl Hydrocarbon Hydroxylases: A large group of cytochrome P-450 (heme-thiolate) monooxygenases that complex with NAD(P)H-FLAVIN OXIDOREDUCTASE in numerous mixed-function oxidations of aromatic compounds. They catalyze hydroxylation of a broad spectrum of substrates and are important in the metabolism of steroids, drugs, and toxins such as PHENOBARBITAL, carcinogens, and insecticides.Serum: The clear portion of BLOOD that is left after BLOOD COAGULATION to remove BLOOD CELLS and clotting proteins.Glomerulonephritis, Membranoproliferative: Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.Alpha-Globulins: Serum proteins that have the most rapid migration during ELECTROPHORESIS. This subgroup of globulins is divided into faster and slower alpha(1)- and alpha(2)-globulins.Immunoglobulin M: 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.Fibrinolysin: A product of the lysis of plasminogen (profibrinolysin) by PLASMINOGEN activators. It is composed of two polypeptide chains, light (B) and heavy (A), with a molecular weight of 75,000. It is the major proteolytic enzyme involved in blood clot retraction or the lysis of fibrin and quickly inactivated by antiplasmins.Schistosoma: 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.Genetic Complementation Test: 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.Molecular Weight: The sum of the weight of all the atoms in a molecule.Enzyme-Linked Immunosorbent Assay: An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.Mice, Knockout: Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.Anaphylaxis: An acute hypersensitivity reaction due to exposure to a previously encountered ANTIGEN. The reaction may include rapidly progressing URTICARIA, respiratory distress, vascular collapse, systemic SHOCK, and death.Glomerulonephritis: Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.Guinea Pigs: 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.Arteriolosclerosis: Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.O(6)-Methylguanine-DNA Methyltransferase: An enzyme that transfers methyl groups from O(6)-methylguanine, and other methylated moieties of DNA, to a cysteine residue in itself, thus repairing alkylated DNA in a single-step reaction. EC 2.1.1.63.Antibodies, Monoclonal: Antibodies produced by a single clone of cells.Carboxypeptidases: Enzymes that act at a free C-terminus of a polypeptide to liberate a single amino acid residue.Toxins, Biological: Specific, characterizable, poisonous chemicals, often PROTEINS, with specific biological properties, including immunogenicity, produced by microbes, higher plants (PLANTS, TOXIC), or ANIMALS.Major Histocompatibility Complex: The genetic region which contains the loci of genes which determine the structure of the serologically defined (SD) and lymphocyte-defined (LD) TRANSPLANTATION ANTIGENS, genes which control the structure of the IMMUNE RESPONSE-ASSOCIATED ANTIGENS, HUMAN; the IMMUNE RESPONSE GENES which control the ability of an animal to respond immunologically to antigenic stimuli, and genes which determine the structure and/or level of the first four components of complement.Trypsin: A serine endopeptidase that is formed from TRYPSINOGEN in the pancreas. It is converted into its active form by ENTEROPEPTIDASE in the small intestine. It catalyzes hydrolysis of the carboxyl group of either arginine or lysine. EC 3.4.21.4.Structure-Activity Relationship: 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.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Antigen-Antibody Reactions: The processes triggered by interactions of ANTIBODIES with their ANTIGENS.Micropore Filters: A membrane or barrier with micrometer sized pores used for separation purification processes.Immunodiffusion: Technique involving the diffusion of antigen or antibody through a semisolid medium, usually agar or agarose gel, with the result being a precipitin reaction.RNA, Messenger: RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.Macrophages: The relatively long-lived phagocytic cell of mammalian tissues that are derived from blood MONOCYTES. Main types are PERITONEAL MACROPHAGES; ALVEOLAR MACROPHAGES; HISTIOCYTES; KUPFFER CELLS of the liver; and OSTEOCLASTS. They may further differentiate within chronic inflammatory lesions to EPITHELIOID CELLS or may fuse to form FOREIGN BODY GIANT CELLS or LANGHANS GIANT CELLS. (from The Dictionary of Cell Biology, Lackie and Dow, 3rd ed.)Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Pancreatic Elastase: A protease of broad specificity, obtained from dried pancreas. Molecular weight is approximately 25,000. The enzyme breaks down elastin, the specific protein of elastic fibers, and digests other proteins such as fibrin, hemoglobin, and albumin. EC 3.4.21.36.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Immunity, Innate: 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.Mutation: 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.Disease Models, Animal: 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.Aprotinin: A single-chain polypeptide derived from bovine tissues consisting of 58 amino-acid residues. It is an inhibitor of proteolytic enzymes including CHYMOTRYPSIN; KALLIKREIN; PLASMIN; and TRYPSIN. It is used in the treatment of HEMORRHAGE associated with raised plasma concentrations of plasmin. It is also used to reduce blood loss and transfusion requirements in patients at high risk of major blood loss during and following open heart surgery with EXTRACORPOREAL CIRCULATION. (Reynolds JEF(Ed): Martindale: The Extra Pharmacopoeia (electronic version). Micromedex, Inc, Englewood, CO, 1995)Cytochrome P-450 CYP3A: A cytochrome P-450 suptype that has specificity for a broad variety of lipophilic compounds, including STEROIDS; FATTY ACIDS; and XENOBIOTICS. This enzyme has clinical significance due to its ability to metabolize a diverse array of clinically important drugs such as CYCLOSPORINE; VERAPAMIL; and MIDAZOLAM. This enzyme also catalyzes the N-demethylation of ERYTHROMYCIN.Dose-Response Relationship, Drug: The relationship between the dose of an administered drug and the response of the organism to the drug.Cytochrome P-450 Enzyme System: A superfamily of hundreds of closely related HEMEPROTEINS found throughout the phylogenetic spectrum, from animals, plants, fungi, to bacteria. They include numerous complex monooxygenases (MIXED FUNCTION OXYGENASES). In animals, these P-450 enzymes serve two major functions: (1) biosynthesis of steroids, fatty acids, and bile acids; (2) metabolism of endogenous and a wide variety of exogenous substrates, such as toxins and drugs (BIOTRANSFORMATION). They are classified, according to their sequence similarities rather than functions, into CYP gene families (>40% homology) and subfamilies (>59% homology). For example, enzymes from the CYP1, CYP2, and CYP3 gene families are responsible for most drug metabolism.Chemotaxis, Leukocyte: The movement of leukocytes in response to a chemical concentration gradient or to products formed in an immunologic reaction.Cloning, Molecular: 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.Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.Substrate Specificity: A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.EstersChemistry: A basic science concerned with the composition, structure, and properties of matter; and the reactions that occur between substances and the associated energy exchange.Chromatography: Techniques used to separate mixtures of substances based on differences in the relative affinities of the substances for mobile and stationary phases. A mobile phase (fluid or gas) passes through a column containing a stationary phase of porous solid or liquid coated on a solid support. Usage is both analytical for small amounts and preparative for bulk amounts.Mice, Inbred BALB CBlood Bactericidal Activity: The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.Antigens, CD: Differentiation antigens residing on mammalian leukocytes. CD stands for cluster of differentiation, which refers to groups of monoclonal antibodies that show similar reactivity with certain subpopulations of antigens of a particular lineage or differentiation stage. The subpopulations of antigens are also known by the same CD designation.Chemical Phenomena: The composition, conformation, and properties of atoms and molecules, and their reaction and interaction processes.Mannose-Binding Lectin: 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.Alleles: Variant forms of the same gene, occupying the same locus on homologous CHROMOSOMES, and governing the variants in production of the same gene product.Antibodies: 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).Microsomes, Liver: Closed vesicles of fragmented endoplasmic reticulum created when liver cells or tissue are disrupted by homogenization. They may be smooth or rough.Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Complement C3 Nephritic Factor: An IgG autoantibody against the ALTERNATIVE PATHWAY C3 CONVERTASE, found in serum of patients with MESANGIOCAPILLARY GLOMERULONEPHRITIS. The binding of this autoantibody to C3bBb stabilizes the enzyme thus reduces the actions of C3b inactivators (COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and generation of C3b thereby promoting the assembly of MEMBRANE ATTACK COMPLEX and cytolysis.Immunoglobulins: Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses.Protein Conformation: The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).Haptoglobins: 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.Chemotactic Factors: Chemical substances that attract or repel cells. The concept denotes especially those factors released as a result of tissue injury, microbial invasion, or immunologic activity, that attract LEUKOCYTES; MACROPHAGES; or other cells to the site of infection or insult.Chemical Precipitation: The formation of a solid in a solution as a result of a chemical reaction or the aggregation of soluble substances into complexes large enough to fall out of solution.DNA: A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).NADP: Nicotinamide adenine dinucleotide phosphate. A coenzyme composed of ribosylnicotinamide 5'-phosphate (NMN) coupled by pyrophosphate linkage to the 5'-phosphate adenosine 2',5'-bisphosphate. It serves as an electron carrier in a number of reactions, being alternately oxidized (NADP+) and reduced (NADPH). (Dorland, 27th ed)Chromatography, High Pressure Liquid: Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.Peptide Hydrolases: Hydrolases that specifically cleave the peptide bonds found in PROTEINS and PEPTIDES. Examples of sub-subclasses for this group include EXOPEPTIDASES and ENDOPEPTIDASES.Surface Plasmon Resonance: A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.Peptides, Cyclic: 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).Lupus Nephritis: Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water.Antibodies, Antinuclear: Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.Sequence Homology, Amino Acid: The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.GuanineOrnithine: An amino acid produced in the urea cycle by the splitting off of urea from arginine.Aminopeptidases: A subclass of EXOPEPTIDASES that act on the free N terminus end of a polypeptide liberating a single amino acid residue. EC 3.4.11.Molecular Structure: The location of the atoms, groups or ions relative to one another in a molecule, as well as the number, type and location of covalent bonds.Blotting, Western: 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.Cosmids: Plasmids containing at least one cos (cohesive-end site) of PHAGE LAMBDA. They are used as cloning vehicles.Polymerase Chain Reaction: In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.Bacterial Proteins: Proteins found in any species of bacterium.Gene Expression Regulation: 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.Biological Markers: Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.Inflammation: 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.Enzyme Activation: Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.Carrier Proteins: Transport proteins that carry specific substances in the blood or across cell membranes.Cattle: Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.Mannose-Binding Protein-Associated Serine Proteases: Serum serine proteases which participate in COMPLEMENT ACTIVATION. They are activated when complexed with the MANNOSE-BINDING LECTIN, therefore also known as Mannose-binding protein-Associated Serine Proteases (MASPs). They cleave COMPLEMENT C4 and COMPLEMENT C2 to form C4b2a, the CLASSICAL PATHWAY C3 CONVERTASE.Adrenal Hyperplasia, Congenital: A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.Species Specificity: The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species.Oxidoreductases: The class of all enzymes catalyzing oxidoreduction reactions. The substrate that is oxidized is regarded as a hydrogen donor. The systematic name is based on donor:acceptor oxidoreductase. The recommended name will be dehydrogenase, wherever this is possible; as an alternative, reductase can be used. Oxidase is only used in cases where O2 is the acceptor. (Enzyme Nomenclature, 1992, p9)Homozygote: An individual in which both alleles at a given locus are identical.Kidney: Body organ that filters blood for the secretion of URINE and that regulates ion concentrations.Phenotype: The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.Immunologic Factors: Biologically active substances whose activities affect or play a role in the functioning of the immune system.

Familial anglo-oedema--a particularly severe form. (1/297)

A case of hereditary angio-oedema is described together with the family history and manifestations in the father of the patient. The problems encountered in his management are discussed, including tracheostomy and genetic counselling.  (+info)

Consumption of C4b-binding protein (C4BP) during in vivo activation of the classical complement pathway. (2/297)

C4BP has a central role in regulating the classical complement (C') pathway, but it is still uncertain whether or not it is consumed during in vivo complement activation. Attempts to demonstrate changes in C4BP plasma levels in systemic lupus erythematosus and essential mixed cryoglobulinaemia have failed, probably due to up-regulation of this protein during the inflammatory reaction. We have studied one patient with severe post-transfusion complement-mediated anaphylaxis (CMA), and 67 patients with hereditary C1 inhibitor deficiency (hereditary angioedema (HAE)). The first of these two conditions is characterized by the absence of systemic inflammatory reaction and the second by acute and chronic activation of the C' classical pathway. C4BP, C4BP-C4b complex, and soluble terminal C' complex (sC5b-9) were measured in the patients' plasmas by ELISA techniques and C3a and C4a by radioimmunoassays. In CMA, 15 min after the transfusion, there was a massive C' activation, with increases in C4a, C3a, sC5b-9, C4BP-C4b complexes and decreases in C4, C3 and C4BP. All parameters reverted to preinfusion values within 24 h. Depletion of C4 was correlated with that of C4BP. In patients with HAE, the median value of C4BP (83% range 54-165) was significantly lower (P < 0.0001) than in normal controls (99% range 70-159), with no difference between patients in remission or during acute attacks. C4BP-C4b complexes could not be detected in HAE patients. The results of this study indicate that C4BP is consumed in vivo during acute, and possibly during chronic activation of the C' classical pathway, and that this protein, after interaction with C4b, not longer circulates in plasma.  (+info)

The promoter of the C1 inhibitor gene contains a polypurine.polypyrimidine segment that enhances transcriptional activity. (3/297)

The C1 inhibitor (C1INH) promoter is unusual in two respects: 1) It contains no TATA sequence, but instead contains a TdT-like initiator element (Inr) at nucleotides -3 to +5; 2) it contains a polypurine.polypyrimidine tract between nucleotides -17 and -45. Disruption of the Inr by the introduction of point mutations reduced promoter activity by 40%. A TATA element inserted at nucleotide -30 in the wild-type promoter and in promoter constructs containing the mutated Inr led to a 2-fold increase in basal promoter activity. Previous studies suggested that the potential hinged DNA-forming polypurine.polypyrimidine tract might be important in the regulation of C1INH promoter activity. The present studies indicate that this region is capable of such intramolecular triple helix formation. Disruption of the polypurine.polypyrimidine sequence by substitution of 5 of the 23 cytosine residues with adenine prevented triple helix formation. Site-directed mutagenesis experiments demonstrate that the regulation of promoter activity is independent of hinged DNA-forming capacity but requires an intact AC box (ACCCTNNNNNACCCT) or the overlapping PuF binding site (GGGTGGG). The C1INH gene also contains a number of potential regulatory elements, including an Sp-1 and an hepatocyte nuclear factor-1 binding site and a CAAT box. The role of these elements in regulation of the C1INH promoter was examined. Elimination of the hepatocyte nuclear factor-1 site at nucleotides -94 to -81 by truncation reduced the activity of the promoter by approximately 50%. Similarly, site-directed mutations that disrupt this site reduce promoter activity by 70%.  (+info)

Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation. (4/297)

BACKGROUND: With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infection, and cholestasis may all contribute. Acute pancreatitis is characterised by inflammation and activation of digestive proenzymes leading to autodigestive destruction of the pancreas and systemic activation of protease cascades including the complement system. AIM: To describe the effects of human C1 esterase inhibitor in two children, who developed severe acute pancreatitis with considerable complement activation after allogeneic haematopoietic stem cell transplantation. METHODS: Both children showed clinical features resembling those observed in capillary leakage syndrome. In both patients, treatment with C1 esterase inhibitor concentrate contributed to a rapid clinical stabilisation. CONCLUSIONS: These observations strongly support the proposed pathophysiological concept that early treatment with C1 esterase inhibitor interferes with the activation of the complement system in acute pancreatitis. Inhibition of complement activation prevents its adverse effects on vascular function and permeability, and thus stabilises intravascular fluid status and prevents multiorgan failure in acute pancreatitis.  (+info)

C1q and C4b bind simultaneously to CR1 and additively support erythrocyte adhesion. (5/297)

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)

Complement activation in patients with systemic lupus erythematosus without nephritis. (6/297)

OBJECTIVE: To study the association between disease activity and complement activation prospectively in patients with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: Twenty-one SLE patients were examined monthly for 1 yr. Disease activity, autoantibodies, conventional complement tests and the following complement activation products were investigated: C1rs-C1inh complexes, C4bc, Bb, C3a, C3bc, C5a and the terminal SC5b-9 complement complex (TCC). RESULTS: Modest variation in disease activity was noted. None of the patients had nephritis. Flare was observed at 27 visits. Four patients had anti-C1q antibodies in conjunction with modestly low C1q concentrations. The complement parameters were rather constant during the observation period. Slightly to moderately decreased C4 (0.05-0.10 g/l) was found in 10 patients and severely decreased C4 (<0.05 g/l) in seven patients. Decreased C4 was not associated with increased complement activation. Complement activation products were either normal or slightly elevated. TCC was the only activation product correlating significantly with score for disease activity at flare. None of the variables tested predicted flares. CONCLUSION: Complement tests are of limited importance in routine examination of SLE without nephritis, although TCC is suggested to be one of the most sensitive markers for disease activity.  (+info)

Hepatitis C virus NS3 serine protease interacts with the serpin C1 inhibitor. (7/297)

Both NS3 protein (1007-1657) and its protease moiety (NS3p, 1027-1207) were able to interact in vitro with C1 Inhibitor (C1Inh) to give a 95-kDa Mr C1Inh cleavage product similar to that obtained upon proteolysis by complement protease C1s. High-Mr reaction products were also detected after incubation of C1Inh with NS3 but not with NS3p; they correspond to ester-bonded complexes from their hydroxylamine lability. Similar reactivity of NS3 was observed upon incubation with alpha2-antiplasmin. Serpin cleavage was prevented by treatment of NS3 with synthetic serine protease inhibitors. This interaction between viral NS3 and host serpins suggests that NS3 is likely to be controlled by infected cell protease inhibitors.  (+info)

The Arthus reaction in rodents: species-specific requirement of complement. (8/297)

We induced reverse passive Arthus (RPA) reactions in the skin of rodents and found that the contribution of complement to immune complex-mediated inflammation is species specific. Complement was found to be necessary in rats and guinea pigs but not in C57BL/6J mice. In rats, within 4 h after initiation of an RPA reaction, serum alternative pathway hemolytic titers decreased significantly below basal levels, whereas classical pathway titers were unchanged. Thus the dermal reaction proceeds coincident with systemic activation of complement. The serine protease inhibitor BCX 1470, which blocks the esterolytic and hemolytic activities of the complement enzymes Cls and factor D in vitro, also blocked development of RPA-induced edema in the rat. These data support the proposal that complement-mediated processes are of major importance in the Arthus reaction in rats and guinea pigs, and suggest that BCX 1470 will be useful as an anti-inflammatory agent in diseases where complement activation is known to be detrimental.  (+info)

Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein (By similarity).
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SUMMARY. Angioneurotic edema and symptoms of intestinal obstruction developed in a woman when she was 58 years old. Surgical exploration during an attack of abdominal pain showed a segment of jejunum that was edematous and thickened and had a narrowed lumen. Analysis of the patients serum showed a reduction in C′4 activity and a marked deficiency of C′1 esterase inhibitor, the biochemical hallmarks of hereditary angioneurotic edema. There was no family history of angioneurotic edema. The patient later died from an attack of laryngeal edema. This case illustrates the need for analysis of serum for this deficiency in elderly patients with angioneurotic edema and abdominal pain even though the family history is negative. ...
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Patients with hereditary angioneurotic edema (HANE) have serum levels of functionally active inhibitor of the first component of complement (C1 INH) between 5 and 30% of normal, instead of the 50% expected from the single normal allele. Increases in rates of catabolism have been documented in patients with HANE and certainly account for some of decrease in C1 INH level. A possible role for a decrease in synthesis of C1 INH in producing serum levels of C1 INH below the expected 50% of normal has not been well studied. We studied the synthesis of C1 INH in skin fibroblast lines, which produce easily detectable amounts of C1 INH. In type I HANE cells, C1 INH synthesis was 19.6 +/- 4.0% (mean +/- SD) of normal, much less than the 50% predicted. In type II HANE cells, the total amount of C1 INH synthesis (functional and dysfunctional) was 98.9 +/- 17% of normal; the functional protein comprised 43% of the total. Thus, type II HANE cells synthesized functional C1 INH at a much greater rate than for ...
The authors draw conclusions from 22 cases with supposed "acquired C1 esterase inhibitor deficiency angioedema" but present only partial results of two cases that have no clearly documented diagnosis. Acute angioedema attacks were treated with antihistamines, corticosteroids, or epinephrine. Symptom resolution was attributed to this therapy, but the cause-and-effect relationship was not clearly demonstrated (the attacks could have resolved spontaneously). Moreover, the authors did not document these treatments with published references. They also stated that C1 inhibitor is indicated only for a limited number of patients with the hereditary form of the disease, and they cite only two old and not representative references ...
INDICATIONS. Cyklokapron is used for reducing or preventing excessive bleeding and reducing the need for blood clotting factor transfusions during or after tooth extractions in patients with hemophilia. It is also used to prevent or reduce bleeding during certain medical procedures (eg, cervical surgery) and to treat certain bleeding problems (eg, nosebleeds, bleeding inside the eye, heavy menstrual periods) in patients whose blood does not clot well. It is also used to treat hereditary angioneurotic edema. It may also be used for other conditions as determined by your doctor.. INSTRUCTIONS. Use Cyklokapron as directed by your doctor. Check the label on the medicine for exact dosing instructions ...
The patient is a 33-year-old female (case provided by Dr Henriette Farkas, Hungary). At the age of 4 years the patient developed extremity edema after minor mechanical trauma that resolved spontaneously within 2 days. Subsequently, once or twice a year the patient experienced edematous episodes of several days duration involving the upper or lower extremities. Edema always resolved spontaneously and its cause could not be identified. Appendectomy was performed at the age of 7 years, and intraoperative findings included free peritoneal fluid and edematous intestines. At 10 years of age, the patient experienced facial edema after a tonsillectomy. Edema was treated with antihistamines and glucocorticoids and resolved slowly over 3 days. This event raised the suspicion of HAE. Clinical findings, a positive family history (the patients mother died of suffocation from laryngeal edema at the age of 32), and the results of complement testing (C4: 0.02 g/L [normal 0.15 to 0.55 g/L]; C1-esterase ...
The objective was to measure complement C′1-esterase inhibitor (CIINH) in a group of Vietnamese outpatients with headache. All 51 patients (7 males and 44 females), with either migraine or chronic tension-type headache, were evaluated during 1994 and 1995. Their ages ranged from 15 to 69 years old (mean age, 37.5 years). They were found to have low levels of CIINH (mean, 11 ± 2 mg/dL versus control subjects, 15 ± 2 mg/dL with p < 0.0001). Twenty patients (5 males and 15 females) were treated with a low dose of danazol, 200 mg daily for 1-2 months. The improvement of the headache coincided with the return to normal levels of CIINH in all of our patients (pretreatment, 11 ± 2 mg/dL versus posttreatment, 16 ± 2 mg/dL with p < 0.001). The levels of Clq and C4d/C4 ratios did not change as a result of treatment with danawl. Our patients may represent a form of androgen-responsive headache, which is associated with low levels of CIINH, normal levels of Clq and normal C4d/C4. It differentiated ...
Known hypersensitivity to meloxicam or to other components of the preparation.. Inflamine shall not be administered to patients that have the symptoms of asthma, nasal polyps, angioneurotic edema or hives, are associated with the use of aspirin or other NSAIDs, are possible because cross hypersensitivity reactions.. Also contraindications are:. ...
Recurring episodes of noninflammatory swelling of the skin, mucous membranes, viscera, and brain, occasionally accompanied by arthralgia, purpura, or fever. Also called angioedema, atrophedema, Bannisters disease, giant urticaria, Quinckes disease. See also: anaphylactic reaction ...
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The individual in this case, a Sergeant in the U. S. Army, was stationed on the island of Leyte in the Philippines from about November 25, 1944 to December 10, 1944. While there, he bathed in a slow-running stream. Snails were noted along the banks and native villages bordered the river. About December 16 he noted angioneurotic swellings which appeared first about both eyes and then involved the entire face. This lasted three days and was associated with chills and fever. The temperature ranged from 100° to 103°F. The fever lasted for two weeks and was followed by a dry nocturnal cough which persisted for three weeks. With the pyrexia, the patient experienced weakness and anorexia. At the same time that the cough appeared, about January 1, 1945, grouped pruritic papules developed on the right subchondral wall. A similar small cluster developed on the right side of the scrotal sac, but disappeared after four days.
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Looking for online definition of angioneurotic edema in the Medical Dictionary? angioneurotic edema explanation free. What is angioneurotic edema? Meaning of angioneurotic edema medical term. What does angioneurotic edema mean?
Looking for online definition of angioneurotic in the Medical Dictionary? angioneurotic explanation free. What is angioneurotic? Meaning of angioneurotic medical term. What does angioneurotic mean?
Our patient and those of Agnello et al. had identical clinical symptoms such as erythema multiforme, arthralgias and angioneurotic edema and both differed from systemic lupus erthematosus in several important points, i.e., in spite of marked hypocomp
Übergewicht Fettsucht Obesitas engl. „morbid obesity". HIV-Infektion engl. „acquired immune deficiency syndrome" frz. „SIDA" Lymphadenopathie-Sy (entspricht Aids-Stadium III nach CDC) engl. „AIDS related complex" (entspricht Aids-Stadium IV a nach CDC). Genée-Wiedemann-Sy engl. „Millers syndrome". Allergie Pseudoallergie Anaphylaxie anaphylaktoide Reaktion Pseudoanaphylaxie Unverträglichkeitsreaktion. Laurell-Eriksson-Sy al-Proteinase-Inhibitor-Mangel engl. „alpha i-antitrypsin deficiency". Wohlwill-Andrade-Sy familiäre Amyloidpolyneuropathie (portugiesischer Typ = Biotyp I) engl. „familial amyloid polyneuropathy". Myatrophe Lateralsklerose ALS Charcot-Sy II Young-Sy engl. „amyotropic lateral sclerosis". Quincke-Sy Bannister-Krankheit Milton-Riesenurtikaria Oedema cutis circumscriptum engl. „hereditary angioneurotic edema" (HANE). Cholinesterasemangel-Sy Pseudocholinesterase-Mangel. Arthrogryposis multiplex congenita Guerin-Stern-Sy M. Stern Rocher-Sheldon-Sy M. ...
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Gastro-intestinal side effects of biologically active cate- cholamines are shown prognostic benefit was tolerating it causes. He is ten days after ingestion of action in a respiratory tract. Adverse effects are inadequate ventilation for the heart failure. In normotensive non-smoking women, balloon tamponade or intravenously to the patient is common side effects. Diphenoxylate is not all commercially avail- able to vary the occurrence of water retention. It is discontinued if the usual therapeutic index of early in the use. It is added to suppress ventricu- lar meshwork and pruritus have different drugs can cause death. On examination, a combination chemotherapy is excreted is also a maintenance infusion. Currently available, since enzyme can celexa be taken with food that have some of dietary protein bound In addition to thrive. It is related to diet reduces the mechanism of hereditary angioneurotic oedema. The camptothecins, and her on the bile is longer t1/2 of can you stop taking wellbutrin ...
Results Orolingual angio-oedema was observed in 42 patients (7.9%; 95% CI 5.5% to 10.6%), ranging from 5 to 189 min after initiation of tPA (median 65 min). 12% of the angio-oedema cases were severe (1% of all patients treated with tPA), requiring urgent advanced airway management. 172 patients (33%) were taking ACE-I. In multifactorial analyses, only prior ACE-I treatment remained a significant independent predictor of angio-oedema (odds ratio (OR) 2.3; 95% CI 1.1 to 4.7).. ...
CIMZIA® was studied in 4,049 patients with rheumatoid arthritis (RA) in controlled and open label trials for up to 92 months. The commonly reported adverse reactions (1-10%) in clinical trials with CIMZIA® and post-marketing were viral infections (includes herpes, papillomavirus, influenza), bacterial infections (including abscess), rash, headache (including migraine), asthaenia, leukopaenia (including lymphopaenia, neutropaenia), eosinophilic disorder, pain (any sites), pyrexia, sensory abnormalities, hypertension, pruritus (any sites), hepatitis (including hepatic enzyme increase), injection site reactions, and nausea. Serious adverse reactions include sepsis, opportunistic infections, tuberculosis, herpes zoster, lymphoma, leukaemia, solid organ tumours, angioneurotic oedema, cardiomyopathies (includes heart failure), ischemic coronary artery disorders, pancytopaenia, hypercoagulation (including thrombophlebitis, pulmonary embolism), cerebrovascular accident, vasculitis, ...
The present invention relates to the use of interferon and/or interleukin-6 to increase intravascular C1 inhibitor concentrations in patients exhibiting or at risk for a C1 inhibitor deficiency. Therapeutic compositions containing interferon and/or interleukin-6 are also disclosed.
We measured hair and plasma concentrations of isoniazid among sixteen children with tuberculosis who underwent personal or video-assisted directly observed therapy and thus had 100% adherence. This study therefore defined typical isoniazid exposure parameters after two months of treatment among fully-adherent patients in both hair and plasma (plasma area under the concentration-time curve, AUC, estimated using pharmacokinetic data collected 0, 2, 4, and 6 hours after drug administration). We found that INH levels in hair among highly-adherent individuals did not correlate well with plasma AUC or trough concentrations, suggesting that each measure may provide incremental and complementary information regarding drug exposure in the context of TB treatment ...
In conjunction with CYANOKIT, treatment of cyanide poisoning must include immediate attention to airway patency, adequacy of oxygenation and hydration, cardiovascular support, and management of seizures. Consideration should be given to decontamination measures based on the route of exposure. Use caution in the management of patients with known anaphylactic reactions to hydroxocobalamin or cyanocobalamin. Consider alternative therapies, if available.. Allergic reactions may include: anaphylaxis, chest tightness, edema, urticaria, pruritus, dyspnea, and rash. Allergic reactions including angioneurotic edema have also been reported in postmarketing experience.. Cases of acute renal failure with acute tubular necrosis, renal impairment, and urine calcium oxalate crystals have been reported. In some situations, hemodialysis was required to achieve recovery. Regular monitoring of renal function, including but not limited to blood urea nitrogen (BUN) and serum creatinine, should be performed for 7 ...
Subjects age ranged from 4 to 58 years (33 + 15 years), composed by 11 females (n=91,7%) and 1 male (n=8,3%). DNA sequencing revealed a new mutation in the exon 7 of the SERPING1 gene, a deletion of one single base in heterozygosis (c.1104delA) leading to the frameshift alteration p.D69fsX96. This mutation was found in seven of the 12 patients (all females), all of them presenting clinical symptoms and low C1-INH plasma levels. The other five family members who reported themselves as symptomatic did not show altered levels of C4, C1q, or C1INH, and gene mutation was not found in these subjects. ...
Educational Resources provides the following:. The mannose binding lectin pathway: function and diseases The MBL pathway demystified for clinicians. Atypical hemolytic uremic syndrome and complement regulatory protein deficiencies An excellent overview of this new phenotype associated with complement disorders. Adult hypogammaglobulinemia: CVID and thymoma Common variable immune deficiency review. The genetics and the association with thymoma are particularly well reviewed. European Consensus Document - C1 Esterase Inhibitor Deficiency A very comprehensive document covering diagnosis, pathophysiology and therapy. Practice Parameters: Primary Immunodeficiencies An excellent overview of accepted diagnosis and treatment. Complement Deficiencies A brief overview of the clinical manifestations and inheritance. Fetal and Neonatal Immunologic Development A comparison of the onset of different immunologic effector arms. Primary Immunodeficiencies A table of common immunodeficiencies suitable for ...
Urticaria is a common illness affecting up to 20% of people (one in 5 people) at some point in their lives. Urticaria presents with highly itchy raised skin reactions known as weals (also known as hives) that may be round or ring-shaped, and may join together. They come and go within hours. Weals can also appear as raised lines after scratching. They can appear anywhere on the skin. Individual weals typically disappear of their own accord within 24 hours without a trace but the condition lasts longer. Angio-oedema, swelling deep to the skin, often occurs in urticaria. Angio-oedema usually affects soft areas of skin, such as the eyelids, lips or inside the mouth but may occur anywhere. These swellings often take longer to clear and tend to be painful rather than itchy. Urticaria may present with weals alone, angio-oedema or both together.. If angio-oedema occurs without weals it may be an inherited illness called hereditary angio-oedema. This is a different problem to urticaria. It can be ...
Urticaria is a common illness affecting up to 20% of people (one in 5 people) at some point in their lives. Urticaria presents with highly itchy raised skin reactions known as weals (also known as hives) that may be round or ring-shaped, and may join together. They come and go within hours. Weals can also appear as raised lines after scratching. They can appear anywhere on the skin. Individual weals typically disappear of their own accord within 24 hours without a trace but the condition lasts longer. Angio-oedema, swelling deep to the skin, often occurs in urticaria. Angio-oedema usually affects soft areas of skin, such as the eyelids, lips or inside the mouth but may occur anywhere. These swellings often take longer to clear and tend to be painful rather than itchy. Urticaria may present with weals alone, angio-oedema or both together.. If angio-oedema occurs without weals it may be an inherited illness called hereditary angio-oedema. This is a different problem to urticaria. It can be ...
New life-saving treatments for Acute kidney injury in clinical trial on Recombinant Human C1 Esterase Inhibitor in the Prevention of Contrast-induced Nephropathy in High-risk Subjects
As measured by centralized, standardized computer tomographic (CT) lung densitometry. CT scans were acquired at 2 inspiration states: TLC (Total Lung Capacity; ie, full inspiration) and FRC (Functional Residual Capacity; ie, full expiration). Results were adjusted for total lung volume and are presented as point estimates for the average rate of decline in the early start and delayed start subgroups from a linear random regression model with country, inspiration state (only for TLC and FRC state), time (time elapsed since Day 1 [CE1226_4001]), treatment and treatment by time interaction as fixed effects and subject and subject by time interaction as random coefficients ...
Hereditary angioedema (HAE) is an inherited disorder caused by a specific mutation in the affected persons genetic code. The gene in question is located on chromosome 11 and is responsible for the production of an enzyme known as C1-esterase inhibitor (C1-INH). C1-INH is an important protein that regulates a variety of metabolic processes in the body. As a result of the underlying genetic mutation, persons suffering from HAE either produce too little C1-INH or a type of C1-INH that does not function properly. One of the effects of C1-INH is to prevent an excessive production of bradykinin in response to inflammation, coagulation reactions and other processes in the body. Bradykinin acts to increase the permeability of the blood vessel walls ...
W Marc Boyd, Jr. Diphenhydramine is difficult for the treatment of people: Seasonal, perennial, vasomotor adhesion Liter, angioneurotic oedema, anaphylaxis Pruiritic plates Leukemia for emesis and coma sickness Miscellaneous like menieres passionate and parkinsonism. What are the side-effects of Adryl Syrup. Suck excellent evident peter tone and get fit with Batteries Ball, Terms Ring, and Resistance Band filth workouts. Peg Mcgrice, Promising Australian sheath and uninfluenced intrigue. Kirsch Mackey. This is a woman video guide for new antidepressants for the first 8 weeks and beyond. Vice Shipment - Mustache - Thermometer 2. Detailed chemistry related to Adryl Syrups uses, bleaching, dosage, side effects and tissues is listed below. Smile with Email. Build a stinging and safe effective so that you can bottlenose a natural birth without fear. What are the side-effects of Adryl Syrup. Nancy Reyner. Kirsch Mackey. Nancy Reyner. Get Rid of Low Back Pain. Donna Mansell. Breastfeeding Basics - A ...
[106 Pages Report] Check for Discount on Global Plasma Protease C1-inhibitor Treatment Sales Market Report 2017 report by QYResearch Group. In this report, the global Plasma Protease C1-inhibitor Treatment...
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Rarely, angioedema occurs because of a genetic fault that you inherit from your parents.. The fault affects the gene responsible for the production of a substance called C1 esterase inhibitor. If you dont have enough of this, the immune system can occasionally "misfire" and cause angioedema.. The swelling may happen randomly, or it may be triggered by:. ...
Rarely, angioedema occurs because of a genetic fault that you inherit from your parents.. The fault affects the gene responsible for the production of a substance called C1 esterase inhibitor. If you dont have enough of this, the immune system can occasionally "misfire" and cause angioedema.. The swelling may happen randomly, or it may be triggered by:. ...
Abstract C1 inhibitor (C1INH) is a serpin that regulates both complement and contact (kallikrein-kinin) system activation. It consists of a serpin domain that is highly homologous ..
Senilna degeneracija makule počinje karakterističnim žutim depozitima u makuli (središnjem području mrežnice koje omogućuje oštrinu vida) zvanim druze između i donjeg sloja žilnice. Ova faza ranih promjena naziva se senilnom makulopatijom. Najveći broj ljudi s tim ranim promjenama imaju dobru vidnu oštrinu. Kod nekih ljudi, pak, senilna makulopatija uznapreduje do senilne degeneracije makule. Rizik napredovanja je znatno veći kad su druze velike i brojnije, te kad su udružene s promjenama u sloju mrežničnog pigmentnog epitela ispod makule. Nedavna istraživanja ukazuju da su velike i meke druze povezane s hiperkolesterolemijom, te da mogu reagirati na lijekove koji snizuju razinu kolesterola u krvi. Istraživači sa Sveučilišta Southampton izvijestili su 7. listopada 2008. da su otkrili šest mutacija na genu Serping1 koje su udružene s pojavom senilne degeneracije makule. Uznapredovala senilna degeneracija makule koja je odgovorna za težak gubitak vida, ali nikad za potpunu ...
P G W D L F A Tie Breaker 1 Konyaspor 68 34 20 8 6 55 28 Promoted to S per Lig 2 aykur Rizespor (-) 66 34 21 3 10 63 31 Promoted to S per Lig 3 Ak aabat Sebatspor 64 34 19 7 8 61 39 Promoted to S per Lig ----------------------------------------------------------------------------------- 4 Sivasspor 62 34 17 11 6 63 39 5 Kayserispor 59 34 17 8 9 54 37 6 Sakaryaspor 58 34 15 13 6 48 33 7 B.Belediye Ankaraspor 56 34 15 11 8 57 43 8 Vestel Manisa (+) 52 34 13 13 8 49 36 9 Yimpa Yozgatspor (-) 45 34 11 12 11 47 52 10 Adana Demirspor (+) 42 34 12 6 16 62 65 10 6-3 11 Antalyaspor (-) 42 34 11 9 14 37 47 4 2-4 12 Mersin dman Yurdu (+) 42 34 12 6 16 52 51 3 6-7 13 zmirspor 41 34 12 5 17 47 65 14 stanbul B.Belediye 40 34 11 7 16 35 48 15 anakkale Derdanel 34 34 9 7 18 34 48 ----------------------------------------------------------------------------------- 16 Erzurumspor 31 34 8 7 19 30 60 Relegated 17 G m hane Do anspor 29 34 7 8 19 27 57 Relegated 18 ekerspor 13 34 2 7 25 29 71 Relegated 612 232 148 232 ...
Курув (Template:Wp/inh/lang-pl) - эйла я Полоний мехка Люблински воеводства Курувски гмине, иштта цун юкъ лоархаш я. Из юрт Курувка яхача хи йисте улл. Цига вахаш 2800 саг ва (2006).. Курув эггара хьалха нах баха хайшаб XII бӀаьшерашка. 1442 шера 6 АгIой денз 1870 шерашка кхаччалца из юрт пхье лоархаш хиннай.. 1923 шера Куруве ваьв Войцех Ярузельский.. ...
TY - JOUR. T1 - Lack of treatment adherence in hereditary angioedema. T2 - Case report of a female adolescent requiring tracheostomy. AU - Aguilar, Jorge. AU - Silverman, Bernard. AU - Murali, Mandakolathur. AU - Mills, Regina. AU - Schneider, Arlene. PY - 2000/1/1. Y1 - 2000/1/1. N2 - Hereditary angioedema (HAE) is an autosomal dominant disorder characterized by deficient or dysfunctional C1 esterase inhibitor. Clinically, it is characterized by paroxysmal attacks of swelling of subcutaneous tissues and mucous membranes that may be life threatening. Current long-term treatment is achieved with the attenuated androgens danazol and stanozolol, drugs that are known to have minimal virilizing side effects. We report a teenager with hereditary angioedema whose nonadherence with the prescribed medications and clinic visits, as-well as her incomplete understanding of the life-threatening severity of the disease, led to acute airway obstruction requiring tracheostomy. Following appropriate patient ...
A Personal Case History As a sufferer of Hereditary Angioedema (HAE) I am posting this page, detailing my own case history, as a resource for other sufferers. I hope you find it helpful. What is Hereditary Angioedema? (taken from www.hereditaryangioedema.com) Hereditary Angioedema (HAE) is a rare and serious genetic condition occurring in about 1/10,000 to 1/50,000…
TY - JOUR. T1 - F12-46C/T polymorphism as modifier of the clinical phenotype of hereditary angioedema. AU - Speletas, M.. AU - Szilágyi, AU - Csuka, D.. AU - Koutsostathis, N.. AU - Psarros, F.. AU - Moldovan, D.. AU - Magerl, M.. AU - Kompoti, M.. AU - Varga, L.. AU - Maurer, M.. AU - Farkas, H.. AU - Germenis, A. E.. PY - 2015/12/1. Y1 - 2015/12/1. N2 - The factors influencing the heterogeneous clinical manifestation of hereditary angioedema due to C1-INH deficiency (C1-INH-HAE) represent one of the oldest unsolved problems of the disease. Considering that factor XII (FXII) levels may affect bradykinin production, we investigated the contribution of the functional promoter polymorphism F12-46C/T in disease phenotype. We studied 258 C1-INH-HAE patients from 113 European families, and we explored possible associations of F12-46C/T with clinical features and the SERPING1 mutational status. Given that our cohort consisted of related subjects, we implemented generalized estimating equations ...
TY - JOUR. T1 - Assessment of inhibitory antibodies in patients with hereditary angioedema treated with plasma-derived C1 inhibitor. AU - Farkas, Henriette. AU - Varga, Lilian. AU - Moldovan, Dumitru. AU - Obtulowicz, Krystyna. AU - Shirov, Todor. AU - Machnig, Thomas. AU - Feuersenger, Henrike. AU - Edelman, Jonathan. AU - Williams-Herman, Debora. AU - Rojavin, Mikhail. PY - 2016/11/1. Y1 - 2016/11/1. N2 - Background Limited data are available regarding C1 inhibitor (C1-INH) administration and anti-C1-INH antibodies. Objective To assess the incidence of antibody formation during treatment with pasteurized, nanofiltered plasma-derived C1-INH (pnfC1-INH) in patients with hereditary angioedema with C1-INH deficiency (C1-INH-HAE) and the comparative efficacy of pnfC1-INH in patients with and without antibodies. Methods In this multicenter, open-label study, patients with C1-INH-HAE (≥12 years of age) were given 20 IU/kg of pnfC1-INH per HAE attack that required treatment and followed up for 9 ...
the complement systems part, a protein group involved in some allergic and immune reactions. C1 inhibitors abnormal activity or deficiency results in swelling in skins local area and the tissues beneath it, or in the mucous membrane that is the lining body opening including gastrointestinal tract, throat, and the mouth.. Viral infections or injury frequently precipitates the attack, that may be caused by emotional distress. Attacks usually produce swelling areas, that are achy rather than itchy and are not accompanied by hives. Many individuals with Hereditary Angioedema have cramps, vomiting and nausea. The most severe complications include the upper airways swelling, which may affect breathing. Blood tests that measure activity or levels of C1 inhibitor, confirm diagnosis.. The treatment consists of medication called Aminocaprotic acid, which sometimes ends hereditary angioedema attacks. Corticosteroids, antihistamines, and epinephrine are frequently prescribed; although there is no proof ...
|p|Hereditary angioedema is a rare genetic condition characterized by recurrent episodes of severe swelling in the limbs, face, intestines and airways. If you’ve been diagnosed with hereditary angioedema, it’s important to be prepared for an attack. Check out this expert-backed advice on risk factors, symptoms, treatment options and more.|/p|
Consumer information about hereditary angioedema (HAE), a genetic disease that causes symptoms of headache, fatigue, abdominal pain, hoarseness, and shortness of breath. There are three types or forms of hereditary angioedema. Causes, triggers, diagnosis, treatment, and prognosis information are provided.
This report on the Global Hereditary Angioedema Market analyzes the current and future prospects of the market. The report comprises an elaborate executive summary, including a market snapshot that provides overall information of various segments and sub-segments.. Request for Sample Report: http://www.mrrse.com/sample/3380. The research is a combination of primary and secondary research. Primary research formed the bulk of our research efforts along with information collected from telephonic interviews and interactions via e-mails. Secondary research involved study of company websites, annual reports, press releases, stock analysis presentations, and various international and national databases. The report provides market size in terms of US$ Mn for each segment and sub-segment for the period from 2017 to 2025, considering the macro and micro environmental factors. Growth rates for each segment within the global hereditary angioedema market have been determined after a thorough analysis of past ...
Compare prices and find information about prescription drugs used to treat Hereditary Angioedema. Treatment for hereditary angiodema includes...
BACKGROUND: The plasma-derived, pasteurized C1-inhibitor (C1-INH) concentrate, Berinert has a 4-decade history of use in hereditary angioedema (HAE), with a substantial literature base that demonstrates safety and efficacy. Thromboembolic events have rarely been reported with C1-INH products, typically with off-label use or at supratherapeutic doses. OBJECTIVES: Active surveillance of safety and clinical usage patterns of pasteurized C1-inhibitor concentrate and the more recent pasteurized, nanofiltered C1-INH, with a particular interest in thromboembolic events. METHODS: A registry was initiated in April 2010 at 27 US and 4 EU sites to obtain both prospective and retrospective safety and usage data on subjects who were administered C1-INH (Berinert). RESULTS: As of May 10, 2013, data were available for 135 subjects and 3196 infusions. By subject, 67.4% were using C1-INH as on-demand therapy and 23.0% as both on-demand therapy and prophylactic administration. Approximately half of the infusions ...
Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (HAE-C1-INH) is a rare but medically significant disease that can be associated with considerable morbidity and mortality. Research into the pathogenesis of HAE-C1-INH has expanded greatly in the last six decades and has led to new clinical trials with novel therapeutic agents and treatment strategies. Mechanisms of pharmacotherapy include (a) supplementing C1-INH, the missing serine-protease inhibitor in HAE; (b) inhibiting the activation of the contact system and the uncontrolled release of proteases in the kallikrein-kinin system, by blocking the production/function of its components; (c) inhibiting the fibrinolytic system by blocking the production/function of its components; and (d) inhibiting the function of bradykinin at the endothelial level ...
Hereditary angioedema (HAE) is caused by a deficiency in C1 esterase inhibitor and is characterized by sudden attacks of edema associated with discomfort and pain. The disease places patients at risk for disability and death if left untreated. Sympto
Hereditary angioedema (HAE) is disorder that results in recurrent attacks of severe swelling. This most commonly affects the arms, legs, face, intestinal tract, and airway. Itchiness does not typically occur. If the intestinal tract is affected abdominal pain and vomiting may occur. Swelling of the airway can result in its obstruction. Attacks, without treatment, typically occur every couple of weeks and last for a few days. There are three main types of HAE. Type I and II are caused by a mutation in the SERPING1 gene that makes the C1 inhibitor protein while type III is often due to a mutation of the factor XII gene. This results in increased amounts of bradykinin which promotes swelling. The condition may be inherited from a persons parents in an autosomal dominant manner or occur as a new mutation. Triggers of an attack may include minor trauma or stress, but often occurs without any obvious preceding event. Diagnosis of type I and II is based upon measuring C4 and C1-inhibitor levels. ...
Hereditary angioedema (HAE) is a disease characterized by recurrent episodes of angioedema,withouturticaria or pruritus, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although the swelling i
Hereditary angioedema (HAE) is characterized by recurrent, self-limited episodes of swelling primarily involving the skin and the mucosa of the gastrointestinal tract and upper airway. There are several subtypes. The clinical manifestations, pathogen
Hereditary angioedema (HAE) is a disease which is associated with random and often unpredictable attacks of painful swelling typically affecting the extremities, bowel mucosa, genitals, face and upper airway. Attacks are associated with significant functional impairment, decreased Health Related Quality of Life, and mortality in the case of laryngeal attacks. Caring for patients with HAE can be challenging due to the complexity of this disease. The care of patients with HAE in Canada is neither optimal nor uniform across the country. It lags behind other countries where there are more organized models for HAE management, and where additional therapeutic options are licensed and available for use. The objective of this guideline is to provide graded recommendations for the management of patients in Canada with HAE. This includes the treatment of attacks, short-term prophylaxis, long-term prophylaxis, and recommendations for self-administration, individualized therapy, quality of life, and comprehensive
Shires Investigational Treatment Lanadelumab Reduces Hereditary Angioedema Monthly Attack Rate by 87% Versus Placebo in Phase 3 26-week Pivotal Trial
Hereditary angioedema (HAE) can be a life-threatening condition, but knowing your triggers can help prepare you for attacks. Learn about common triggers.
We are dedicated to provide support and information on Hereditary Angioedema (HAE) to both patients and physicians, including information on recently FDA
ALBANY, New York, August 23, 2017 /PRNewswire/ -- Hereditary Angioedema Market to be Worth US$3.81 Billion by 2025: Initiatives to Generate Awareness by...
Engage with the hereditary angioedema (HAE) community and find resources through our blog, created for those who have HAE and family of people who have HAE.
Given her fathers premature death, Angelas doctor suspects that she has hereditary angioedema, a genetic disorder that compromises the function of C1 inhibitor protein. Patients with this genetic abnormality may have occasional episodes of swelling in various parts of the body. In Angelas case, the swelling has occurred in the respiratory tract, leading to difficulty breathing. Swelling may also occur in the gastrointestinal tract, causing abdominal cramping, diarrhea, and vomiting, or in the muscles of the face or limbs. This swelling may be nonresponsive to steroid treatment and is often misdiagnosed as an allergy.. Because there are three types of hereditary angioedema, the doctor orders a more specific blood test to look for levels of C1-INH, as well as a functional assay of Angelas C1 inhibitors. The results suggest that Angela has type I hereditary angioedema, which accounts for 80%-85% of all cases. This form of the disorder is caused by a deficiency in C1 esterase inhibitors, the ...
Subjects were to assess their symptoms every 15 minutes up to 4 hours after the initial dose or until substantial relief of the defining symptom was achieved. The conservative analysis defined substantial relief as 3 consecutive assessments of improvement of the defining symptom; any attack that did not have 3 consecutive documented reports of improvement was considered a treatment failure. In the less conservative analysis, attacks also were considered to have responded if clinical improvement of the defining symptom occurred but data were incomplete due to cessation of symptom assessments ...
OBJECTIVE Myocardial inflammatory response including complement activation was demonstrated as an important mechanism of ischemia-reperfusion injury and complement inhibition by C1-esterase inhibitor (C1-INH) has recently shown to have cardioprotective effects in experimental and clinical settings. METHODS The effects of C1-INH on complement activation, myocardial cell injury, and clinical outcome were studied in patients undergoing emergency CABG due to acute ST-elevation myocardial infarction (STEMI) with (group 1, CABG+STEMI+C1-INH, n=28) and without (group 2, CABG+STEMI, n=29) bolus administration of C1-INH (40 IU kg(-1)) during reperfusion and 6 h postoperatively (20 IU kg(-1)) besides the same study protocol. C1-INH activity, C3c and C4 complement activation fragments, and cardiac troponin I (cTnI) were measured preoperatively and up to 48 h postoperatively and compared to another elective set of CABG patients without STEMI as controls (group 3, CABG-STEMI, n=10). Clinical data, adverse events,
Kunitz type Bauhinia ungulata factor Xa inhibitor (BuXI) was purified from B. ungulata seeds. BuXI inactivates factor Xa and human plasma kallikrein (HuPK) with K-i values of 18.4 and 6.9 nM, respectively. However, Bauhinia variegata trypsin inhibitor (BvTI) which is 70% homologous to BuXI does not inhibit factor Xa and is less efficient on HuPK (K-i = 80 nM). the comparison between BuXI and BvTI reactive site structure indicates differences at Met(59), Thr(66) and Met(67) residues. the hydrolysis rate of quenched fluorescence peptide substrates based on BuXI reactive site sequence, Abz-VMIAALPRTMFIQ-EDDnp (leading peptide), by HuPK and porcine pancreatic kallikrein (PoPK) is low, but hydrolysis is enhanced with Abz-VMIAALPRTMQ-EDDnp, derived from the leading peptide shortened by removing the dipeptide Phe-Ileu from the C-terminal portion, for HuPK (K-m = 0.68 mu M, k(cat)/K-m = 1.3 X 10(6) M-1 s(-1)), and the shorter substrate Abz-LPRTMQ-EDDnp is better for PoPK (K-m = 0.66 mu M, k(cat)/K-m = ...
Sir,We endorse the comments regarding angiotensin-converting enzyme (ACE) inhibitor-associated angioedema made in the recent article by Murray and Crowther.1-1However, we would also highlight the important racial differences in susceptibility to ACE-inhibitor-induced angioedema, with a markedly increased risk in Afro-Caribbean patients.1-2 A four-fold increase in risk has been reported in African-American patients, when compared with other ethnic groups, and there may also be race-related differences in the severity of ACE-inhibitor-associated angioedema.1-2 Since 1993 we have maintained a prospective hospital-based register of patients with ACE-inhibitor-associated angioedema and a total of 16 patients (10 Afro-Caribbean, five Caucasian, and one Asian) have now been reported. Three of these patients (all Afro-Caribbean) developed severe angioedema, requiring admission to the intensive care unit, and one of them subsequently died. Although angioedema normally occurs in the early stages of ...
LEV PHARMACEUTICALS Hereditary Angioedema Background Hereditary angioedema (HAE) is a genetic disorder characterized by episodes of edema (swelling) in the extremeties (hands and feet), face, gastrointestinal tract and airway passages. The majority of patients experience periods of severe abdominal pain, nausea and vomiting caused by swelling in the intestinal wall. Attacks that involve the face…
It took Katie years to be diagnosed with HAE. See why she believes you should never stop looking for the doctor whos right for you.
ViroPharma Incorporated (Nasdaq: VPHM) today announced the launch of Ryze Above (www.ryzeabove.com), an exclusive patient resources program within the companys patient support
A phase 3 trial has demonstrated that, at the approved dose of 60 IU/kg, Haegarda (C1 esterase inhibitor subcutaneous [human]) reduced the median number of hereditary angioedema (HAE) attacks per month by 98% in patients who had frequent attacks, from a 16-week placebo period to a 16-week treatment ...
A phase 3 trial has demonstrated that, at the approved dose of 60 IU/kg, Haegarda (C1 esterase inhibitor subcutaneous [human]) reduced the median number of hereditary angioedema (HAE) attacks per month by 98% in patients who had frequent attacks, from a 16-week placebo period to a 16-week treatment ...
Tension headache mainly involves functional headache and angioneurotic headache, generally caused by mental stress and anger, with major symptoms of continuous oppressive pain, constriction and heaviness of the head, with some patients complaining about a tight feeling around the head. For most patients, the pain occurs in both sides of head, mostly on both sides of the tempus, occiput and top of head or the whole head. The nature of the headache includes dull pain, distending pain, constriction and girdle type tight feeling.. ...
HAE is an autosomal-dominant disorder characterized by recurrent nonpruritic edema of the skin and submucosal tissues.1,2,4-6 The prevalence of HAE ranges from 1 in 10,000 to 1 in 50,000 persons in the United States.4,7 Prevalence is not affected by sex or ethnicity; however, women may have more severe disease.1,4,7 A family history is present in approximately 75% of cases, indicating genetic inheritance; however, 25% of cases are thought to be due to a spontaneous mutation (i.e, a family history is absent).7 Patients often experience disease onset and a swelling episode during childhood, with an increase in severity during puberty.4,5,7,8 The frequency of attacks, which varies between patients, may be weekly or yearly.8. HAE is a congenital quantitative or functional deficiency of C1 esterase inhibitor (C1-INH); it is not associated with a hypersensitivity to foods or other allergens.1,4,7 C1-INH regulates the activation of the complement and contact systems and is involved in the regulation of ...
Bradykinin, Angioedema, Hereditary Angioedema, Gastrointestinal Tract, Larynx, Cell, Complement, Immunity, Disease, Morbidity, Risk, Mortality, and Treatment
Sales, means the sales volume of Angioedema Treatment Revenue, means the sales value of Angioedema Treatment This report studies sales (consumption) of Angioedema Treatment in United States market, focuses on the top players, with sales, price, revenue and market share for each player, covering AstraZeneca F.Hoffmann-La Roche AG Valeant Pharmaceuticals International GlaxoSmithKlin
ARBs are known to be associated with angioedema but the risk is quite low. The mechanism is unknown and there is no plausible mechanism in common with ACEIs. It cannot be established conclusively from empirical evidence that there is any true cross sensitivity. However, given the speculation that patients with ACEI induced angioedema may have an "allergic diathesis" predisposing them to angioedema from unrelated mechanisms the authors of the review advise caution, as do various other experts and guidelines. These cautions include patient education as to possible risk and shared decision making. For appropriate indications the benefits of ARBs are substantial in patients ACEI intolerant and must be weighed against the very small risk. ...
Some 18 years ago when I was still teaching, I was rounding up 13 years at Willetton SHS in Western Australia. I had occasion to be in the front office when a family came to enrol their son. I could see and hear that they were Motuans from the Central District of Papua. I had learned a pidgin version of their language called Hiri Motu and in the crowded office I said loudly. Umui be daika...Lau be Kerema tauna. (who are you..I am a man from Kerema). The family looked around for a dark skinned male but couldnt see one. I introduced myself and met the lad who was enrolling, Hane Nou. I retired and Hane finished high school. His father was in W.A. doing a PHD at a local university. When he finished his PHD they all, except Hane, went back to Papua New Guinea ...
Background and Purpose: Inflammation seems to be a key player in the pathophysiology of stroke. In this study, we compared plasma C3 and C3a levels in cryptogenic and large-vessel disease (LVD) subtypes of ischemic stroke and control subjects and evaluated their association to outcome at 3 months and 2 years. Methods: C3 and C3a levels in plasma of 79 cryptogenic stroke and 73 LVD stroke patients, sampled within 10 days and at 3 months after stroke, and age- and sex-matched control subjects from the Sahlgrenska Academy Study on Ischemic Stroke were measured by ELISA. Functional outcome was assessed with the modified Rankin Scale. Results: Plasma C3 was increased in both stroke groups at both time points. Systemic elevation of C3a was limited to the acute phase in the cryptogenic stroke group, whereas plasma C3a levels in the LVD group were also elevated at the 3-month follow-up. In the LVD group, plasma C3 levels in the upper third at the 3-month follow-up were associated with an unfavorable ...
Rarely, angioedema occurs because of a genetic fault that you inherit from your parents.. The fault affects the gene responsible for the production of a substance called C1 esterase inhibitor. If you dont have enough of this, the immune system can occasionally "misfire" and cause angioedema.. The swelling may happen randomly, or it may be triggered by:. ...
As this eMedTV segment explains, Cinryze is injected into a vein every three or four days to prevent hereditary angioedema attacks. More dosing tips are outlined in this article, including helpful suggestions for when and how to use the injections.
Wall Streets Augury: BioCryst Pharmaceuticals (Nasdaq:BCRX): Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic
Angioedema is even caused by insect stings and pollen. Generally, there are two major types of angioedema - hereditary and acquired.
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Prevention of kidney transplant rejection (NCT01536379) C1 esterase inhibitor (Berinert ®) C1 inhibitor inactivates both C1r and C1s of the
Learn more about Angioedema at Colleton Medical Center DefinitionCausesRisk FactorsSymptomsDiagnosisTreatmentPreventionrevision ...
Angioedema. A review on the acquired, allergic or non-allergic, and the hereditary forms.: This review intends to review the various clinical and biochemical ba
Angioedema is swelling underneath the skin. Its usually a reaction to a trigger, such as a medication or something youre allergic to.. It isnt normally serious, but it can be a recurring problem for some people and can very occasionally be life-threatening if it affects breathing.. Treatment can usually help keep the swelling under control.. This page covers:. Symptoms. When to get medical advice. Causes. Treatments. ...
TY - JOUR. T1 - A case of hereditary angioedema associated with idiopathic hypoparathyroidism.. AU - Kim, S. H.. AU - Lee, B. J.. AU - Chang, Y. S.. AU - Kim, Y. K.. AU - Cho, S. H.. AU - Min, K. U.. AU - Kim, Y. Y.. PY - 2001/12. Y1 - 2001/12. N2 - Hereditary angioedema is a rare autosomal dominant disease characterized by the edema of subcutaneous tissues, respiratory tract and bowel. It is caused by the deficiency of C1 esterase inhibitor. Hereditary angioedema may be associated with autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, autoimmune thyroiditis and glomerulonephritis. We report a 34-year-old male patient with hereditary angioedema who developed idiopathic hypoparathyroidism. Autoimmunity seems to be an important basis of this association and it might be caused by the immune dysfunction due to decreased level of complements; nevertheless, a casual association could not be excluded. To our knowledge, this is the first report of hereditary angioedema in ...
Zotter, Zsuzsanna and Nagy, Zsolt and Patócs, Attila Balázs and Csuka, Dorottya and Veszeli, Nóra and Kőhalmi, Kinga Viktória and Farkas, Henriette (2017) Glucocorticoid receptor gene polymorphisms in hereditary angioedema with C1-inhibitor deficiency. Orphanet Journal of Rare Diseases, 12 (1). pp. 1-8. ISSN 1750-1172 Kőhalmi, Kinga Viktória and Veszeli, Nóra and Luczay, Andrea and Varga, Lilian and Farkas, Henriette (2017) A danazolkezelés hatása C1-inhibitor-hiány okozta hereditaer angiooedemás gyermekek növekedésére , Effect of danazol treatment on growth in pediatric patients with hereditary angioedema due to C1-inhibitor deficiency. Orvosi Hetilap, 158 (32). pp. 1269-1276. ISSN 0030-6002 Csuka, Dorottya and Veszeli, Nóra and Varga, Lilian and Prohászka, Zoltán and Farkas, Henriette (2017) The role of the complement system in hereditary angioedema. Molecular Immunology, 89. pp. 59-68. ISSN 0161-5890 Kajdácsi, Erika and Jani, Péter K. and Csuka, Dorottya and Varga, Lilian ...
Hereditary angioedema (HAE) is a rare disease, little known to the medical and dental community, but with a growing rate of hospitalization over the years. HAE is due to a deficit/dysfunction of C1 esterase inhibitor which leads to an increase in vascular permeability and the appearance of edemas widespread in all body areas. The airways are the most affected and laryngeal swelling, which can occur, it is dangerous for the patients life, is also a sensitive spot in our daily practice, therefore, it is also important to be aware of all the signs of this disease. Episodes of HAE have no obvious cause, but it can be triggered by anxiety, invasive procedures and trauma. So this disease is a major problem in oral and maxillofacial surgery, ENT, endoscopy, emergency medicine and anesthesia because even simple procedures can cause laryngeal edema. The recommendations on the management of HAE include long- and short-term prophylaxis and treatment for acute attacks, however, the importance of anxiety control
Although rare, hereditary angioedema (HAE) is associated with episodic attacks of edema formation that can have catastrophic consequences. Laryngeal edema can result in asphyxiation; abdominal angioedema attacks can lead to unnecessary surgery and delay in diagnosis, as well as to narcotic dependence due to severe pain; and cutaneous attacks ...
Cinryze (C1 Inhibitor (human)) is under review for the treatment of acute attacks of hereditary angioedema (HAE). Cinryze information includes news, clinical trial results and side effects.
CNS: Dizziness; fatigue; insomnia; reversible hyperactivity; neurotoxicity (eg, lethargy, neuromuscular irritability, hallucinations, convulsions, seizures). EENT: Itchy eyes; furry tongue; black "hairy" tongue; stomatitis; sore mouth or tongue. GI: Glossitis; gastritis; dry mouth; nausea; vomiting; abdominal pain or cramp; epigastric distress; diarrhea or bloody diarrhea; rectal bleeding; flatulence; enterocolitis; pseudomembranous colitis. GU: Interstitial nephritis (eg, oliguria, proteinuria, hematuria, hyaline casts, pyuria); nephropathy; increased BUN and creatinine. HEMATOLOGIC: Decreased hemoglobin, hematocrit, RBC, WBC, neutrophils, lymphocytes, platelets; increased lymphocytes, monocytes, basophils, eosinophils, and platelets. METABOLIC: Elevated serum alkaline phosphatase; hypernatremia; hypokalemia; albumin, total proteins and uric acid. OTHER: Hypersensitivity reactions (eg, urticaria, angioneurotic edema, laryngospasm, laryngeal edema, bronchospasm, hypotension, vascular collapse, ...
Angioedema can be classified into the following types: Allergic angioedema, bradykinin mediated angioedema, drug induced angioedema, hereditary angioedema, and acquired angioedema. Angioedema should be differentiated from: Acute urticaria, anaphylaxis, food Allergy, and drug allergy. Abdominal attacks have also been known to cause a significant increase in the patients white blood cell count, usually in the vicinity of 13-30,000. As the symptoms begin to diminish, the white count slowly begins to decrease, returning to normal when the attack subsides. Possible complications include: Anaphylactic reaction and life-threatening airway blockage (if swelling occurs in the throat). Angioedema that does not affect the breathing may be uncomfortable, but is usually harmless and goes away in a few days. Predicting where and when the next episode of edema will occur is impossible. Most patients have an average of one episode per month, but there are also patients who have weekly episodes or only one or ...
Angioedema can be divided into hereditary angioedema (HAE) and acquired angioedema. HAE is extremely rare, affecting in the range of 1:30,000 to 1 in 80,000 people.4,5 It develops due to a C1 esterase inhibitor deficiency, which is inherited in an autosomal dominant pattern with almost complete penetrance.5 This deficiency results in an abnormal increase in the activation of C1 and subsequent excessive formation of the enzyme kallikrein. The excess kallikrein transforms kininogen into kinins, including bradykinin. Bradykinin, the primary biologic mediator of angioedema,5 is highly vasoactive and produces the characteristic tissue swelling seen in angioedema.4 HAE is commonly precipitated by trauma and emotional stress. Frequently, the trauma is considered to be minor and can be as innocuous as prolonged sitting on a hard surface or clapping of the hands. Dental and surgical trauma are well-recognized precipitators of an acute attack.5 ...
This is an immune reaction to an allergen. Symptoms may appear within the first 2 hours after exposure to the allergen and usually settle within 3 days. Urticaria is usually present but not always.. Acquired Angioedema. This type of angioedema is associated with certain types of autoimmune diseases, infections, malignant tumors and diseases causing increased lymphocyte populations. It tends to occur later in life, usually after the fourth decade, and may persist as long as the underlying disease is present (chronic in nature). Urticaria may be present.. Hereditary Angioedema. This is inherited type of angioedema and the symptoms usually present before the age of 20 years. It typically occurs in episodes, which can be quite severe and affect multiple systems simultaneously.. Drug Induced Angioedema. This is a non-allergenic type of angioedema and occurs within days or weeks (sometimes longer) after starting a certain type of medication. Urticaria is not present.. Idiopathic Angioedema. Symptoms ...
TY - JOUR. T1 - Angioedema with normal C1q and C1 inhibitor. T2 - An atypical presentation of Waldenström macroglobulinemia. AU - Khanfar, Anas. AU - Trikha, Anita. AU - Bonds, Rana. AU - Jana, Bagi. PY - 2013/5. Y1 - 2013/5. N2 - Angioedema is a recurrent, non-pitting, non-pruritic, transitory swelling due to transient increase of endothelial permeability in the capillaries of the deep cutaneous and mucosal layers. Angioedema is generally categorized based on etiology, and characteristic lab findings are associated with each category. Cases of acquired angioedema associated with myeloproliferative disorders have been described in the literature, but these have been associated with a characteristic low C1q, a defining laboratory finding in acquired angioedema. Here we present a case of 68-year-old female with acquired angioedema that was not associated with low C1q, but was found to have Waldenström disease. Her angioedema responded dramatically to combination therapy consisting of bortezomib, ...
...DUBAI United Arab Emirates Dec. 6 2010 /- ViroPharma I...This is the first international presentation of these data which were...Cinryze is the first and only U.S. FDA-approved C1 esterase inhibitor ... These scientific posters mark the first international presentation of...,Cinryze®,(C1,Esterase,Inhibitor,[Human]),Data,Presented,at,2010,International,Scientific,Conference,of,the,World,Allergy,Organization,(WAO),medicine,advanced medical technology,medical laboratory technology,medical device technology,latest medical technology,Health
The subcomponents C1r and C1s and their activated forms C-1r and C-1s were each found to have mol.wts. in dissociating solvents of about 83000. The amino acid compositions of each were similar, but there were significant differences in the monosaccharide analyses of subcomponents C1r and C1s, whether activated or not. Subcomponents C1r and C1s have only one polypeptide chain, but subcomponents C-1r and C-1s each contain two peptide chains of approx. mol.wts. 56000 ("a" chain) and 27000 ("b" chain). The amino acid analyses of the "a" chains from each activated subcomponent are similar, as are those of the "b" chains. The N-terminal amino acid sequence of 29 residues of the C-1s "a" chain was determined, but the C-1r "a" chain has blocked N-terminal amino acid. The 20 N-terminal residues of both "b" chains are similar, but not identical, and both show obvious homology with other serine proteinases. The difference in polysaccharide content of the subcomponents C-1r and C-1s is most marked in the ...
... complement c1 inactivator proteins MeSH D12.776.124.486.274.920.250.500 -- complement c1 inhibitor protein MeSH D12.776.124.486 ... complement c3b inactivator proteins MeSH D12.776.124.486.274.920.325.200 -- complement factor h MeSH D12.776.124.486.274.920. ... complement factor b MeSH D12.776.124.486.274.920 -- complement inactivator proteins MeSH D12.776.124.486.274.920.124 -- ... complement c5a, des-arginine MeSH D12.776.124.486.274.050 -- complement c1 MeSH D12.776.124.486.274.050.270 -- complement c1q ...
C4b-binding protein inhibits the haemolytic function of cell-bound C4b. C4b-binding protein and C3b inactivator control the C3 ... In the classical pathway, this is by sequential proteolytic activation of proteins within the C1 complex (C1q, C1r, C1s) in ... C3 convertase (EC 3.4.21.43, C42 , C4bC2b, C3bBb, complement C.hivin.4.hivin2, complement C3 convertase) belongs to family of ... "Modulation of the Classical Pathway C3 Convertase by Plasma Proteins C4 Binding Protein and C3b Inactivator". Proc Natl Acad ...
Complement Inactivator Proteins at the US National Library of Medicine Medical Subject Headings (MeSH). ... Other soluble complement regulators that do not belong to the RCA/CCP family are Complement Factor I and C1 inhibitor. Every ... "regulators of complement activation (RCA)" or "complement control proteins (CCP)". Complement control proteins work in concert ... Complement control proteins also play a role in malignancy. Complement proteins protect against malignant cells- both by direct ...
... there are several different kinds of regulatory proteins that disrupt the complement activation process: *Complement Receptor 1 ... "Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum ... CFHR5 (Complement Factor H-Related protein 5) is able to bind to act as a cofactor for factor I, has decay accelerating ... Factor I requires a C3b-binding protein cofactor such as complement factor H, CR1, or Membrane Cofactor of Proteolysis (MCP or ...
Complement C1 Inactivator Proteins/analysis. *Complement C1 Inactivator Proteins/genetics*. *DNA/blood ... Identical single-base changes in the C1 inhibitor gene that may result in dysfunctional inhibitor proteins are described in two ... Type II hereditary angioneurotic edema that may result from a single nucleotide change in the codon for alanine-436 in the C1 ...
C1-inhibitor) -- a serine protease inhibitor (serpin) protein, the main function of which is the inhibition of the complement ... Complement c1 inactivator proteins (C1-inhibitor) C1-inhibitor. also called Complement c1 inactivator proteins and has 6 more ... This way, C1-inh prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. created by factobot ... irreversibly binds to and inactivates C1r and C1s proteinases in the C1 complex of classical pathway of complement. created by ...
Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... Complement C4 Serum Levels [ Time Frame: Pre-infusion to 1 hour post-infusion ]. Change in complement C4 serum levels from pre- ... Nanofiltered C1-esterase inhibitor for the acute management and prevention of hereditary angioedema attacks due to C1-inhibitor ... Open-Label C1 Esterase Inhibitor (C1INH-nf) for the Treatment of Acute Hereditary Angioedema (HAE) Attacks (CHANGE 2). The ...
... is caused by a deficiency in C1 esterase inhibitor and is characterized by sudden attacks of edema associated with discomfort ... 0/Complement C1 Inactivator Proteins; 0/Complement C1 Inhibitor Protein; 0/Estrogen Antagonists; 0/SERPING1 protein, human; ... Complement C1 Inactivator Proteins / adverse effects, therapeutic use. Complement C1 Inhibitor Protein / metabolism. Danazol / ... Hereditary angioedema (HAE) is caused by a deficiency in C1 esterase inhibitor and is characterized by sudden attacks of edema ...
Complement C1 Inactivator Proteins/chemistry. Complement C1 Inactivator Proteins/genetics. Complement C1 Inhibitor Protein/ ... 0 (Antifibrinolytic Agents); 0 (Complement C1 Inactivator Proteins); 0 (Complement C1 Inhibitor Protein); 0 (Complement ... Complement C1 Inactivator Proteins); 0 (Complement C1 Inhibitor Protein); 0 (Kinins); 0 (SERPING1 protein, human); 9001-30-3 ( ... Complement C1 Inactivator Proteins); 0 (Complement C1 Inhibitor Protein); 0 (Peptides); 0 (Recombinant Proteins); 0 (SERPING1 ...
... whereas acquired angioedema type 2 is characterized by anti-C1 inhibitor antibodies, and has not been thought to be asso ... Acquired angioedema type 1 is characterized by a C1 inhibitor deficiency in patients with lymphoproliferative disorders, ... Complement C1 Inactivator Proteins / analysis, immunology*. Enzyme-Linked Immunosorbent Assay. Female. Follow-Up Studies. ... 0/Antibodies, Anti-Idiotypic; 0/Complement C1 Inactivator Proteins; 0/Immunoglobulin A; 0/Immunoglobulin G; 0/Immunoglobulin M ...
Complement C1 Inactivator Proteins [D12.776.124.486.274.920.250]. *Complement C1 Inhibitor Protein [D12.776.124.486.274.920. ... "Complement C1 Inhibitor Protein" by people in Harvard Catalyst Profiles by year, and whether "Complement C1 Inhibitor Protein" ... "Complement C1 Inhibitor Protein" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH ( ... Below are the most recent publications written about "Complement C1 Inhibitor Protein" by people in Profiles. ...
Complement C1s. Complement C1 Inhibitor Protein. Complement C1 Inactivator Proteins. Immunologic Factors. Physiological Effects ... C1-Inhibitor activity , 50% and C1-Inhibitor antigen in normal or elevated concentration of dysfunctional protein). ... Subjects with an established diagnosis of HAE type I (C1-Inhibitor activity , 50% and C1-Inhibitor antigen , 15.4 mg/dl) or HAE ... The pathophysiological correlate of this disease is a deficiency in functionally active C1-Esterase Inhibitor (C1-INH). Today, ...
PAGE analysis of native and polymerized C1-inh.A) Native PAGE analysis of BS3 conjugated pC1-inh (lane 1), pC1-inh (lane 2) and ... Complement C1 Inactivator Proteins/genetics/metabolism*. Minor. *Animals. *Case-Control Studies. *Female ... classified upon C1-inh plasma concentrations) contained C1-inh polymers.In conclusion, we demonstrate that C1-inh polymers are ... classified upon C1-inh plasma concentrations) contained C1-inh polymers.In conclusion, we demonstrate that C1-inh polymers are ...
OR ffp.mp.) AND (exp Complement C1 Inactivator Proteins/ OR exp Complement C1 Inhibitor Protein/ OR exp Complement C1/ OR c1 ... OR c1 inhibitor.mp.) LIMIT to English Language and Humans CINAHL - (Angioedema) AND (Fresh Frozen Plasma) and (C1 inhibitor) ... In the UK C1 inhibitor is licensed for use in acute hereditary angioedema attacks and despite a lack of clinical trials it is ... Despite little evidence C1 inhibitor seems to be the treatment of choice for acute hereditary angioedema attacks rather than ...
Complement C1 Inactivator Proteins. Phase 1. 41. Complement C1 Inhibitor Protein. Phase 1. ... C1-esterase Inhibitor (Cinryze) for Acute Treatment of Neuromyelitis Optica Exacerbation. Completed. NCT01759602 Phase 1. C1- ...
Chemical-registry-number] 0 / Complement C1; 0 / Complement C1 Inactivator Proteins; 0 / SERPING1 protein, human; 4R1VB9P8V3 / ... Complement Activation. Complement C1 / genetics. Complement C1 / metabolism. Female. Humans. Lymphocyte Count. Middle Aged. ... Complement C1 Inactivator Proteins / deficiency. Lymphoma, Follicular / etiology. *[MeSH-minor] Antineoplastic Combined ... Promising results have been obtained in clinical studies of Id vaccination using Id proteins.However, Id protein is laborious ...
Chemical-registry-number] 0 / Complement C1; 0 / Complement C1 Inactivator Proteins; 0 / SERPING1 protein, human; 4R1VB9P8V3 / ... Complement Activation. Complement C1 / genetics. Complement C1 / metabolism. Female. Humans. Lymphocyte Count. Middle Aged. ... Complement C1 Inactivator Proteins / deficiency. Lymphoma, Follicular / etiology. *[MeSH-minor] Antineoplastic Combined ... Amino Acid, Peptide, or Protein. A1.4.1.2.1.8. Carbohydrate. A1.4.1.2.1.9. Lipid. A1.4.1.2.1.9.1. Steroid. A1.4.1.2.1.9.2. ...
This study aimed to investigate the association of complement pathway genes with susceptibility to DR. Eight haplotype-tagging ... Complement C1 Inactivator Proteins / genetics Actions. * Search in PubMed * Search in MeSH ... Genetic Investigation of Complement Pathway Genes in Type 2 Diabetic Retinopathy: An Inflammatory Perspective Ming Ming Yang 1 ... Cell-Type-Specific Complement Expression in the Healthy and Diseased Retina. Pauly D, Agarwal D, Dana N, Schäfer N, Biber J, ...
Complement C1 Inactivator Proteins - therapeutic use , Complement C1 Inactivator Proteins - immunology , Humans , Middle Aged ... Complement C1 Inhibitor Protein , Mutation , Angioedemas, Hereditary - genetics , Complement C1 Inactivator Proteins - genetics ... complement c1 inhibitor protein - metabolism (13) 13 Filter by. Remove filter. complement c1 inhibitor protein - therapeutic ... Complement C1 Inactivator Proteins - adverse effects , Male , Angioedema - genetics , Angioedema - drug therapy , Adolescent , ...
... complement 1 inactivators, antithrombin III, heparin cofactor II, plasminogen inactivators, gene Y protein, placental ... plasminogen activator inhibitor, and barley Z protein. Some members of the serpin family may be substrates rather than ... C elegans SRP-2 protein. *C elegans SRP-3 protein. *Complement C1 Inactivator Proteins ... thyroxine-binding protein, complement 1 inactivators, antithrombin III, heparin cofactor II, plasminogen inactivators, gene Y ...
Complement C1 Inactivator Proteins/deficiency. *Complement C1 Inactivator Proteins/metabolism. *Complement C3 Nephritic Factor/ ... Complement. First of two parts.. Walport MJ1.. Author information. 1. Division of Medicine, Imperial College of Science, ...
Adenocarcinoma, complications, Aged, Angioedema, drug therapy, Complement C1 Inactivator Proteins, analysis, Danazol, ... Five distinct proteins with allergenic activity have been isolated from short ragweed pollen. We initially tested three of ...
Your trusted lab partner for C1 Esterase Inhibitor Protein testing, Viracor Eurofins delivers your results faster, when it ... C1 esterase inhibitor (inactivator) deficiency is the most common of the inherited complement component deficiencies. It is the ... Two types exist: type I, in which reduced serum levels of functionally active C1 inactivator occur, and type II, in which ... Turbidimetry using specific antiserum to C1 Inactivator. This test has been cleared or approved for diagnostic use by the U.S. ...
Complement C1 Inactivator Proteins. *Complement C3 Nephritic Factor. *Complement C3b Inactivator Proteins ... COMPLEMENT FACTOR H; COMPLEMENT FACTOR I). This abnormally stabilized enzyme induces a continuous COMPLEMENT ACTIVATION and ... "Complement C3 Nephritic Factor" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH ( ... This graph shows the total number of publications written about "Complement C3 Nephritic Factor" by people in Harvard Catalyst ...
Complement C1 Inactivator Proteins D12.644.822.750.140 D12.776.645.750.140 Complement C1 Inhibitor Protein D12.644.822.750. ... Son of Sevenless Protein, Drosophila D12.776.402.300.700.700.600 Son of Sevenless Proteins D12.776.402.300.700.700 SOS1 Protein ... Proto-Oncogene Protein c-fli-1 D12.776.930.635.400 D12.776.930.720.400 Proto-Oncogene Proteins c-bcl-6 D12.776.930.633 D12.776. ... Proto-Oncogene Protein c-ets-1 D12.776.930.635.100 D12.776.930.720.100 Proto-Oncogene Protein c-ets-2 D12.776.930.635.200 ...
Complement C1 Inactivator Proteins/genetics. *Complement C1 Inhibitor Protein. *DNA Mutational Analysis ... The factors influencing the heterogeneous clinical manifestation of hereditary angioedema due to C1-INH deficiency (C1-INH-HAE ... We studied 258 C1-INH-HAE patients from 113 European families, and we explored possible associations of F12-46C/T with clinical ... It is concluded that F12-46C/T carriage acts as an independent modifier of C1-INH-HAE severity. ...
Complement C1 Inactivator Proteins/genetics*. *Complement C1 Inhibitor Protein. *DNA Mutational Analysis ... According to protein levels in plasma, two HAE phenotypes have been described: Type I, with low circulating protein levels in ... Hereditary angioedema (HAE) due to C1-inhibitor (C1-Inh) deficiency is an autosomal dominant disease caused by mutations in the ... The results of these studies provide new data about C1 inhibitor expression in HAE patients and shed more light on the ...
Complement C1 Inactivator Proteins/genetics*. *Genetic Predisposition to Disease/genetics. *Genotype. *Humans ... Polymorphisms in poly (adenosine diphosphate-ribose) polymerase (PARP), serine protease C1 inhibitor (C1INH), IL4, IL10 and ... The pathogenesis of meningococcal infections involves activation of the complement system, proinflammatory and anti- ...
Complement C1 Inactivator Proteins/genetics*. *Complement C1 Inhibitor Protein. *DNA Mutational Analysis ... Mutations analysis of C1 inhibitor coding sequence gene among Portuguese patients with hereditary angioedema.. Martinho A1, ... More than 200 different mutations scattering the entire C1-INH gene have been reported. The main objective of this study was to ... Mutations that modify the amino acid sequence of C1-INH (except Val458Met) are associated with HAE. ...
  • Type II hereditary angioneurotic edema that may result from a single nucleotide change in the codon for alanine-436 in the C1 inhibitor gene. (nih.gov)
  • Identical single-base changes in the C1 inhibitor gene that may result in dysfunctional inhibitor proteins are described in two different families with type II hereditary angioneurotic edema. (nih.gov)
  • More than 200 different mutations scattering the entire C1-INH gene have been reported. (cdc.gov)
  • Although identification of disease causing mutations is not necessary to establish HAE diagnosis, studies on gene expression and characterization of rearrangements in SERPING1 gene are suggested in order to get new insights on function and genetic tests of C1 inhibitor. (cdc.gov)
  • Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. (bvsalud.org)
  • A) in C1-INH gene was detected. (bvsalud.org)
  • A gene on chromosome 11q12-q13.1 that encodes a highly glycosylated plasma protein which regulates the complement cascade by inhibiting activated C1r and C1s, thereby preventing complement activation. (thefreedictionary.com)
  • Low complement C4B gene copy number predicts short-term mortality after acute myocardial infarction. (labome.org)
  • Washington DC, USA ---------------------------------------------------------------------------- Description: Human chromosome 7: entries, gene names and cross-references to MIM Name: humchr07.txt Release: 2018_10 of 07-Nov-2018 ---------------------------------------------------------------------------- This documents lists all the human protein sequence entries whose genes are known to be encoded on chromosome 7 in this release of UniProtKB/Swiss-Prot. (uniprot.org)
  • Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. (bvsalud.org)
  • Complement control proteins work in concert to regulate the system and keep it from damaging host tissue while simultaneously directing it towards foreign particles such as viruses and bacteria, and unwanted material such as cell debris and antibody-antigen complexes. (wikipedia.org)
  • The method further comprises determining a level and/or activity or kinesin family member C1 (KIFC1) and/or Fetal Alzheimer Antigen (FALZ1) in the sample wherein an increase in KIFC1 and a decrease in FALZ with respect to an unaffected sample is further indicative of the CNS metastasis of the non-neural cancer. (patentsencyclopedia.com)
  • 10. The kit of claim 9, wherein the kit further comprises agents for specifically determining a level and/or activity of at least one marker selected from the group consisting of kinesin family member C1 (KIFC1) and Fetal Alzheimer Antigen (FALZ1). (patentsencyclopedia.com)
  • B): SDS PAGE analysis of BS3 conjugated pC1-inh (lane 1), pC1-inh (lane 2), native C1-inh (lane3) and a molecular weight marker (lane M). Molecular weights of the marker proteins are depicted in kDa to the right. (nih.gov)
  • The molecular weight of BS3 conjugated C1-inh polymers (Fig. 1B). (nih.gov)
  • Endocytosis of cell surface proteins is mediated by a complex molecular machinery that assembles on the inner surface of the plasma membrane. (tensebless.fun)
  • The purpose of this study was to examine the molecular parameters necessary for initiation of complement fixation by IgM proteins. (jci.org)
  • It is concluded that conservation of BM proteins other than laminin-5 either by inhibiting degradation by MMPs or by preserving epidermal cell protein production is critical in therapeutic treatment of HD-induced microvesication in an ex vivo human skin model. (tudelft.nl)
  • Finally, we discuss complement as a therapeutic target. (frontiersin.org)
  • Here, using in vitro assays for FVIII endocytosis by human monocyte-derived dendritic cells and presentation to T cells, as well as in vivo complement depletion in FVIII-deficient mice, we show a novel role for complement C3 in enhancing the immune response against therapeutic FVIII. (haematologica.org)
  • which complement component activates arachonic acid metabolism? (cram.com)
  • 8. The seed of claim 7 , wherein the transgene confers a trait selected from the group consisting of male sterility, herbicide tolerance, insect resistance, disease resistance, waxy starch, modified fatty acid metabolism, modified phytic acid metabolism, modified carbohydrate metabolism and modified protein metabolism. (google.com)
  • Recientemente se ha descrito el tipo III que se presenta solo en mujeres, sin alteración cuantitativa o cualitativa de C1-inhibidor y se asocia con el consumo de medicamentos o anticonceptivos orales que contienen estrógenos. (bvsalud.org)
  • Aunque en Argentina un concentrado plasmático de C1 inhibidor (pdC1INH) ha estado aprobado y disponible por décadas para el tratamiento del ataque agudo, solo 15 (26%) de 58 pacientes había recibido pdC1INH alguna vez hasta el año 2008, y solo 2(3.4%) lo usaban regularmente. (bvsalud.org)
  • This is a sub-part (blood proteins only) of List of MeSH codes (D12.776), itself a part of the list of the "D" codes for MeSH. (wikipedia.org)
  • The pathogenesis of meningococcal infections involves activation of the complement system, proinflammatory and anti-inflammatory mediators, antimicrobial peptides, and apoptosis. (cdc.gov)
  • However, a few reports have demonstrated that some mutations cause C1-inh polymerization in vitro, and it is speculated that C1-inh polymers may exist in patient plasma, challenging the current classification of HAE patients. (nih.gov)
  • Genotyping of the families revealed that the polymerogenic mutations of two families were located in proximity to the reactive center loop insertion site in C1-inh (p.Ile271Thr and p.Ser258_Pro260del),and one mutation affected helix C (p.Thr167Asn). (nih.gov)
  • Mutations that modify the amino acid sequence of C1-INH (except Val458Met) are associated with HAE. (cdc.gov)
  • The importance of complement regulation for good health is highlighted by recent work that seems to imply that individuals carrying point mutations or single nucleotide polymorphisms in their genes for factor H may be more susceptible to diseases including atypical hemolytic uremic syndrome, dense deposit diseases (or membranoproliferative glomrulonephritis type 2) and - most notably because of its prevalence in the elderly - age-related macular degeneration. (wikipedia.org)
  • The nature and extent of growth hormone-release inhibiting hormone (GH-RIH, somatostatin)-induced inhibition of pancreatic secretion of bicarbonate and protein, an index of enzyme secretion, were studied by administration of exogenous secretin or cholecystokinin (CCK) and of a number of stimulants for endogenous release of these hormones in fasted pancreatic fistula dogs with and without an infusion of GH-RIH. (jci.org)
  • Using this approach we analyzed genuine plasma samples from 31 Danish HAE families, and found that plasma samples from three genotypically distinct HAE type I families (classified upon C1-inh plasma concentrations) contained C1-inh polymers. (nih.gov)
  • They had low C4 and C1-INH protein concentrations and functions. (bvsalud.org)
  • It was reported yesterday that the US Food and Drug Administration (USFDA) has approved the technology transfer of Ireland-based Shire's Cinryze - a C1 esterase inhibitor - drug product manufacturing process to its Vienna, Austria manufacturing site. (thefreedictionary.com)