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 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 C1q: A subcomponent of complement C1, composed of six copies of three polypeptide chains (A, B, and C), each encoded by a separate gene (C1QA; C1QB; C1QC). This complex is arranged in nine subunits (six disulfide-linked dimers of A and B, and three disulfide-linked homodimers of C). C1q has binding sites for antibodies (the heavy chain of IMMUNOGLOBULIN G or IMMUNOGLOBULIN M). The interaction of C1q and immunoglobulin activates the two proenzymes COMPLEMENT C1R and COMPLEMENT C1S, thus initiating the cascade of COMPLEMENT ACTIVATION via the CLASSICAL COMPLEMENT PATHWAY.Complement C5a: The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.Complement Activation: 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 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 C5: C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.Complement C3b: The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.Complement System Proteins: Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).Complement C6: A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.Phosphatidylethanolamine Binding Protein: A ubiquitously found basic protein that binds to phosphatidylethanolamine and NUCLEOTIDES. It is an endogenous inhibitor of RAF KINASES and may play a role in regulating SIGNAL TRANSDUCTION. Phosphatidylethanolamine-binding protein is the precursor of hippocampal cholinergic neurostimulating peptide, which is cleaved from the N-terminal region of the protein.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 C2: A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Complement C9: A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.Receptors, Complement: 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 Membrane Attack Complex: A product of COMPLEMENT ACTIVATION cascade, regardless of the pathways, that forms transmembrane channels causing disruption of the target CELL MEMBRANE and cell lysis. It is formed by the sequential assembly of terminal complement components (COMPLEMENT C5B; COMPLEMENT C6; COMPLEMENT C7; COMPLEMENT C8; and COMPLEMENT C9) into the target membrane. The resultant C5b-8-poly-C9 is the "membrane attack complex" or MAC.Complement C1r: A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.Complement Inactivator Proteins: Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.Complement C7: A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.Complement C3-C5 Convertases: Serine proteases that cleave COMPLEMENT C3 into COMPLEMENT C3A and COMPLEMENT C3B, or cleave COMPLEMENT C5 into COMPLEMENT C5A and COMPLEMENT C5B. These include the different forms of C3/C5 convertases in the classical and the alternative pathways of COMPLEMENT ACTIVATION. Both cleavages take place at the C-terminal of an ARGININE residue.Complement Factor B: 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 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 C1 Inactivator Proteins: Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits.Complement C8: A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.Complement C1: The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.Receptors, Complement 3b: Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.Complement Factor H: An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).Complement C5b: The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.Complement C2a: 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 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.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.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.Receptors, Complement 3d: Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.Anaphylatoxins: Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.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.Proton-Translocating ATPases: Multisubunit enzymes that reversibly synthesize ADENOSINE TRIPHOSPHATE. They are coupled to the transport of protons across a membrane.Mitochondria, Heart: The mitochondria of the myocardium.Angioedema: Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.Submitochondrial Particles: The various filaments, granules, tubules or other inclusions within mitochondria.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.Proteins: Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.Aurovertins: Very toxic and complex pyrone derivatives from the fungus Calcarisporium arbuscula. They bind to and inhibit mitochondrial ATPase, thereby uncoupling oxidative phosphorylation. They are used as biochemical tools.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.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.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.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).Complement C3b Inactivator Proteins: Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.Antigens, CD55: 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.Androgen-Binding Protein: Carrier proteins produced in the Sertoli cells of the testis, secreted into the seminiferous tubules, and transported via the efferent ducts to the epididymis. They participate in the transport of androgens. Androgen-binding protein has the same amino acid sequence as SEX HORMONE-BINDING GLOBULIN. They differ by their sites of synthesis and post-translational oligosaccharide modifications.Carrier Proteins: Transport proteins that carry specific substances in the blood or across cell membranes.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)Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Kinetics: The rate dynamics in chemical or physical systems.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.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.Electrophoresis, Polyacrylamide Gel: Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.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.Steroid 21-Hydroxylase: An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).Complement C3-C5 Convertases, Alternative Pathway: Important enzymes in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. They cleave COMPLEMENT C3 and COMPLEMENT C5.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.Binding Sites: The parts of a macromolecule that directly participate in its specific combination with another molecule.Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Hemolysis: The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.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.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.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.Molecular Weight: The sum of the weight of all the atoms in a molecule.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.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.Placental Hormones: Hormones produced by the placenta include CHORIONIC GONADOTROPIN, and PLACENTAL LACTOGEN as well as steroids (ESTROGENS; PROGESTERONE), and neuropeptide hormones similar to those found in the hypothalamus (HYPOTHALAMIC HORMONES).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.I-kappa B Proteins: A family of inhibitory proteins which bind to the REL PROTO-ONCOGENE PROTEINS and modulate their activity. In the CYTOPLASM, I-kappa B proteins bind to the transcription factor NF-KAPPA B. Cell stimulation causes its dissociation and translocation of active NF-kappa B to the nucleus.Blood Proteins: Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.Ribonuclease, Pancreatic: An enzyme that catalyzes the endonucleolytic cleavage of pancreatic ribonucleic acids to 3'-phosphomono- and oligonucleotides ending in cytidylic or uridylic acids with 2',3'-cyclic phosphate intermediates. EC 3.1.27.5.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.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Mice, Inbred C57BLLupus 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.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 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.Phagocytosis: The engulfing and degradation of microorganisms; other cells that are dead, dying, or pathogenic; and foreign particles by phagocytic cells (PHAGOCYTES).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).Recombinant Proteins: Proteins prepared by recombinant DNA technology.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.Uracil-DNA Glycosidase: An enzyme that catalyzes the HYDROLYSIS of the N-glycosidic bond between sugar phosphate backbone and URACIL residue during DNA synthesis.Plant Proteins: Proteins found in plants (flowers, herbs, shrubs, trees, etc.). The concept does not include proteins found in vegetables for which VEGETABLE PROTEINS is available.Endo-1,4-beta Xylanases: Enzymes which catalyze the endohydrolysis of 1,4-beta-D-xylosidic linkages in XYLANS.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.Protein Structure, Tertiary: The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.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.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.Glycoproteins: Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.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.Adenosine Triphosphatases: A group of enzymes which catalyze the hydrolysis of ATP. The hydrolysis reaction is usually coupled with another function such as transporting Ca(2+) across a membrane. These enzymes may be dependent on Ca(2+), Mg(2+), anions, H+, or DNA.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.Mitochondrial Proton-Translocating ATPases: Proton-translocating ATPases responsible for ADENOSINE TRIPHOSPHATE synthesis in the MITOCHONDRIA. They derive energy from the respiratory chain-driven reactions that develop high concentrations of protons within the intermembranous space of the mitochondria.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).Intracellular Signaling Peptides and Proteins: Proteins and peptides that are involved in SIGNAL TRANSDUCTION within the cell. Included here are peptides and proteins that regulate the activity of TRANSCRIPTION FACTORS and cellular processes in response to signals from CELL SURFACE RECEPTORS. Intracellular signaling peptide and proteins may be part of an enzymatic signaling cascade or act through binding to and modifying the action of other signaling factors.Bacterial Proteins: Proteins found in any species of bacterium.Cholesterol Ester Transfer Proteins: Proteins that bind to and transfer CHOLESTEROL ESTERS between LIPOPROTEINS such as LOW-DENSITY LIPOPROTEINS and HIGH-DENSITY LIPOPROTEINS.Signal Transduction: The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.Phosphoprotein Phosphatases: A group of enzymes removing the SERINE- or THREONINE-bound phosphate groups from a wide range of phosphoproteins, including a number of enzymes which have been phosphorylated under the action of a kinase. (Enzyme Nomenclature, 1992)Phosphorylation: The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.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.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.Adenylyl Imidodiphosphate: 5'-Adenylic acid, monoanhydride with imidodiphosphoric acid. An analog of ATP, in which the oxygen atom bridging the beta to the gamma phosphate is replaced by a nitrogen atom. It is a potent competitive inhibitor of soluble and membrane-bound mitochondrial ATPase and also inhibits ATP-dependent reactions of oxidative phosphorylation.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.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).N-Glycosyl Hydrolases: A class of enzymes involved in the hydrolysis of the N-glycosidic bond of nitrogen-linked sugars.Trypsin Inhibitors: Serine proteinase inhibitors which inhibit trypsin. They may be endogenous or exogenous compounds.Protein Phosphatase 1: A eukayrotic protein serine-threonine phosphatase subtype that dephosphorylates a wide variety of cellular proteins. The enzyme is comprised of a catalytic subunit and regulatory subunit. Several isoforms of the protein phosphatase catalytic subunit exist due to the presence of multiple genes and the alternative splicing of their mRNAs. A large number of proteins have been shown to act as regulatory subunits for this enzyme. Many of the regulatory subunits have additional cellular functions.NF-kappa B: Ubiquitous, inducible, nuclear transcriptional activator that binds to enhancer elements in many different cell types and is activated by pathogenic stimuli. The NF-kappa B complex is a heterodimer composed of two DNA-binding subunits: NF-kappa B1 and relA.Myosin-Light-Chain Phosphatase: A phosphoprotein phosphatase that is specific for MYOSIN LIGHT CHAINS. It is composed of three subunits, which include a catalytic subunit, a myosin binding subunit, and a third subunit of unknown function.Kidney Glomerulus: A cluster of convoluted capillaries beginning at each nephric tubule in the kidney and held together by connective tissue.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.DNA Primers: Short sequences (generally about 10 base pairs) of DNA that are complementary to sequences of messenger RNA and allow reverse transcriptases to start copying the adjacent sequences of mRNA. Primers are used extensively in genetic and molecular biology techniques.Apolipoproteins: Protein components on the surface of LIPOPROTEINS. They form a layer surrounding the hydrophobic lipid core. There are several classes of apolipoproteins with each playing a different role in lipid transport and LIPID METABOLISM. These proteins are synthesized mainly in the LIVER and the INTESTINES.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.Calpain: Cysteine proteinase found in many tissues. Hydrolyzes a variety of endogenous proteins including NEUROPEPTIDES; CYTOSKELETAL PROTEINS; proteins from SMOOTH MUSCLE; CARDIAC MUSCLE; liver; platelets; and erythrocytes. Two subclasses having high and low calcium sensitivity are known. Removes Z-discs and M-lines from myofibrils. Activates phosphorylase kinase and cyclic nucleotide-independent protein kinase. This enzyme was formerly listed as EC 3.4.22.4.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.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.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.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.Hydrolysis: The process of cleaving a chemical compound by the addition of a molecule of water.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.Cyclin-Dependent Kinase Inhibitor p21: A cyclin-dependent kinase inhibitor that mediates TUMOR SUPPRESSOR PROTEIN P53-dependent CELL CYCLE arrest. p21 interacts with a range of CYCLIN-DEPENDENT KINASES and associates with PROLIFERATING CELL NUCLEAR ANTIGEN and CASPASE 3.Protease Inhibitors: Compounds which inhibit or antagonize biosynthesis or actions of proteases (ENDOPEPTIDASES).Arteriolosclerosis: Thickening of the walls of small ARTERIES or ARTERIOLES due to cell proliferation or HYALINE deposition.Time Factors: Elements of limited time intervals, contributing to particular results or situations.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.Calcium-Binding Proteins: Proteins to which calcium ions are bound. They can act as transport proteins, regulator proteins, or activator proteins. They typically contain EF HAND MOTIFS.Antibodies, Monoclonal: Antibodies produced by a single clone of cells.Transfection: The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.Hot Temperature: Presence of warmth or heat or a temperature notably higher than an accustomed norm.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.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.Sequence Alignment: The arrangement of two or more amino acid or base sequences from an organism or organisms in such a way as to align areas of the sequences sharing common properties. The degree of relatedness or homology between the sequences is predicted computationally or statistically based on weights assigned to the elements aligned between the sequences. This in turn can serve as a potential indicator of the genetic relatedness between the organisms.Hydrogen-Ion Concentration: The normality of a solution with respect to HYDROGEN ions; H+. It is related to acidity measurements in most cases by pH = log 1/2[1/(H+)], where (H+) is the hydrogen ion concentration in gram equivalents per liter of solution. (McGraw-Hill Dictionary of Scientific and Technical Terms, 6th ed)Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Blotting, Northern: Detection of RNA that has been electrophoretically separated and immobilized by blotting on nitrocellulose or other type of paper or nylon membrane followed by hybridization with labeled NUCLEIC ACID PROBES.Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.Gene Expression: The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.Protein Kinases: A family of enzymes that catalyze the conversion of ATP and a protein to ADP and a phosphoprotein.Proto-Oncogene Proteins c-raf: A ubiquitously expressed raf kinase subclass that plays an important role in SIGNAL TRANSDUCTION. The c-raf Kinases are MAP kinase kinase kinases that have specificity for MAP KINASE KINASE 1 and MAP KINASE KINASE 2.Crystallography, X-Ray: The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)DNA Glycosylases: A family of DNA repair enzymes that recognize damaged nucleotide bases and remove them by hydrolyzing the N-glycosidic bond that attaches them to the sugar backbone of the DNA molecule. The process called BASE EXCISION REPAIR can be completed by a DNA-(APURINIC OR APYRIMIDINIC SITE) LYASE which excises the remaining RIBOSE sugar from the DNA.Chromatography, Gel: Chromatography on non-ionic gels without regard to the mechanism of solute discrimination.Carbonyl Cyanide p-Trifluoromethoxyphenylhydrazone: A proton ionophore that is commonly used as an uncoupling agent in biochemical studies.Protein Kinase Inhibitors: Agents that inhibit PROTEIN KINASES.Cyclin-Dependent Kinases: Protein kinases that control cell cycle progression in all eukaryotes and require physical association with CYCLINS to achieve full enzymatic activity. Cyclin-dependent kinases are regulated by phosphorylation and dephosphorylation events.Inhibitor of Apoptosis Proteins: A conserved class of proteins that control APOPTOSIS in both VERTEBRATES and INVERTEBRATES. IAP proteins interact with and inhibit CASPASES, and they function as ANTI-APOPTOTIC PROTEINS. The protein class is defined by an approximately 80-amino acid motif called the baculoviral inhibitor of apoptosis repeat.Ribonucleases: Enzymes that catalyze the hydrolysis of ester bonds within RNA. EC 3.1.-.DNA, Complementary: Single-stranded complementary DNA synthesized from an RNA template by the action of RNA-dependent DNA polymerase. cDNA (i.e., complementary DNA, not circular DNA, not C-DNA) is used in a variety of molecular cloning experiments as well as serving as a specific hybridization probe.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.)Macromolecular Substances: Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.Reverse Transcriptase Polymerase Chain Reaction: A variation of the PCR technique in which cDNA is made from RNA via reverse transcription. The resultant cDNA is then amplified using standard PCR protocols.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.Recombinant Fusion Proteins: Recombinant proteins produced by the GENETIC TRANSLATION of fused genes formed by the combination of NUCLEIC ACID REGULATORY SEQUENCES of one or more genes with the protein coding sequences of one or more genes.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.Cyclins: A large family of regulatory proteins that function as accessory subunits to a variety of CYCLIN-DEPENDENT KINASES. They generally function as ENZYME ACTIVATORS that drive the CELL CYCLE through transitions between phases. A subset of cyclins may also function as transcriptional regulators.Up-Regulation: A positive regulatory effect on physiological processes at the molecular, cellular, or systemic level. At the molecular level, the major regulatory sites include membrane receptors, genes (GENE EXPRESSION REGULATION), mRNAs (RNA, MESSENGER), and proteins.Mice, Inbred BALB CLiver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Blood Bactericidal Activity: The natural bactericidal property of BLOOD due to normally occurring antibacterial substances such as beta lysin, leukin, etc. This activity needs to be distinguished from the bactericidal activity contained in a patient's serum as a result of antimicrobial therapy, which is measured by a SERUM BACTERICIDAL TEST.Transcription, Genetic: The biosynthesis of RNA carried out on a template of DNA. The biosynthesis of DNA from an RNA template is called REVERSE TRANSCRIPTION.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.Mitochondria: Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)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.Drug Stability: The chemical and physical integrity of a pharmaceutical product.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).DNA-Binding Proteins: Proteins which bind to DNA. The family includes proteins which bind to both double- and single-stranded DNA and also includes specific DNA binding proteins in serum which can be used as markers for malignant diseases.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.Cyclin-Dependent Kinase Inhibitor p27: A cyclin-dependent kinase inhibitor that coordinates the activation of CYCLIN and CYCLIN-DEPENDENT KINASES during the CELL CYCLE. It interacts with active CYCLIN D complexed to CYCLIN-DEPENDENT KINASE 4 in proliferating cells, while in arrested cells it binds and inhibits CYCLIN E complexed to CYCLIN-DEPENDENT KINASE 2.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.Brain: The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.Dimerization: The process by which two molecules of the same chemical composition form a condensation product or polymer.Swine: Any of various animals that constitute the family Suidae and comprise stout-bodied, short-legged omnivorous mammals with thick skin, usually covered with coarse bristles, a rather long mobile snout, and small tail. Included are the genera Babyrousa, Phacochoerus (wart hogs), and Sus, the latter containing the domestic pig (see SUS SCROFA).Substrate Specificity: A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.Chromatography, High Pressure Liquid: Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.Cysteine Proteinase Inhibitors: Exogenous and endogenous compounds which inhibit CYSTEINE ENDOPEPTIDASES.Apoptosis: One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.Tumor Necrosis Factor-alpha: Serum glycoprotein produced by activated MACROPHAGES and other mammalian MONONUCLEAR LEUKOCYTES. It has necrotizing activity against tumor cell lines and increases ability to reject tumor transplants. Also known as TNF-alpha, it is only 30% homologous to TNF-beta (LYMPHOTOXIN), but they share TNF RECEPTORS.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).Plasmids: Extrachromosomal, usually CIRCULAR DNA molecules that are self-replicating and transferable from one organism to another. They are found in a variety of bacterial, archaeal, fungal, algal, and plant species. They are used in GENETIC ENGINEERING as CLONING VECTORS.

C1-Esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema. (1/83)

C1-esterase inhibitor (C1-Inh) therapy was introduced in clinical medicine about 25 years ago as a replacement therapy for patients with hereditary angioedema caused by a deficiency of C1-Inh. There is now accumulating evidence, obtained from studies in animals and observations in patients, that administration of C1-Inh may have a beneficial effect as well in other clinical conditions such as sepsis, cytokine-induced vascular leak syndrome, acute myocardial infarction, or other diseases. Activation of the complement system, the contact activation system, and the coagulation system has been observed in these diseases. A typical feature of the contact and complement system is that on activation they give rise to vasoactive peptides such as bradykinin or the anaphylatoxins, which in part explains the proinflammatory effects of either system. C1-Inh, belonging to the superfamily of serine proteinase inhibitors (serpins), is a major inhibitor of the classical complement pathway, the contact activation system, and the intrinsic pathway of coagulation, respectively. It is, therefore, endowed with anti-inflammatory properties. However, inactivation of C1-Inh occurs locally in inflamed tissues by proteolytic enzymes (e.g., elastase) released from activated neutrophils or bacteria thereby leading to increased local activation of the various host defense systems. Here we will give an overview on the biochemistry and biology of C1-Inh. We will discuss studies addressing therapeutic administration of C1-Inh in experimental and clinical conditions. Finally, we will provide an explanation for the therapeutic benefit of C1-Inh in so many different diseases.  (+info)

C1 inhibitor cross-linking by tissue transglutaminase. (2/83)

C1 inhibitor, a plasma proteinase inhibitor of the serpin superfamily involved in the regulation of complement classical pathway and intrinsic blood coagulation, has been shown to bind to several components of the extracellular matrix. These reactions may be responsible for C1 inhibitor localization in the perivascular space. In the study reported here, we have examined whether C1 inhibitor could function as a substrate for plasma (factor XIIIa) or tissue transglutaminase. We made the following observations: 1) SDS-polyacrylamide gel electrophoresis and autoradiography showed that C1 inhibitor exposed to tissue transglutaminase (but not to factor XIIIa) incorporated the radioactive amine donor substrate [(3)H]putrescine in a calcium-dependent manner; 2) the maximum stoichiometry for the uptake of [(3)H]putrescine by C1 inhibitor was 1:1; 3) proteolytic cleavage and peptide sequencing of reduced and carboxymethylated [(3)H]putrescine-C1 inhibitor identified Gln(453) (P'9) as the single amine acceptor residue; 4) studies with (125)I-labeled C1 inhibitor showed that tissue transglutaminase was also able to cross-link C1 inhibitor to immobilized fibrin; and 5) C1 inhibitor cross-linked by tissue transglutaminase to immobilized fibrin had inhibitory activity against its target enzymes. Thus, tissue transglutaminase-mediated cross-linking of C1 inhibitor to fibrin or other extracellular matrix components may serve as a mechanism for covalent serpin binding and influence local regulation of the proteolytic pathways inhibited by C1 inhibitor.  (+info)

SERPIN regulation of factor XIa. The novel observation that protease nexin 1 in the presence of heparin is a more potent inhibitor of factor XIa than C1 inhibitor. (3/83)

In the present studies we have made the novel observation that protease nexin 1 (PN1), a member of the serine protease inhibitor (SERPIN) superfamily, is a potent inhibitor of the blood coagulation Factor XIa (FXIa). The inhibitory complexes formed between PN1 and FXIa are stable when subjected to reducing agents, SDS, and boiling, a characteristic of the acyl linkage formed between SERPINs and their cognate proteases. Using a sensitive fluorescence-quenched peptide substrate, the K(assoc) of PN1 for FXIa was determined to be 7.9 x 10(4) m(-)(1) s(-)(1) in the absence of heparin. In the presence of heparin, this rate was accelerated to 1.7 x 10(6), M(-)(1) s(-)(1), making PN1 a far better inhibitor of FXIa than C1 inhibitor, which is the only other SERPIN known to significantly inhibit FXIa. FXIa-PN1 complexes are shown to be internalized and degraded by human fibroblasts, most likely via the low density lipoprotein receptor-related protein (LRP), since degradation was strongly inhibited by the LRP agonist, receptor-associated protein. Since FXIa proteolytically modifies the amyloid precursor protein, this observation may suggest an accessory role for PN1 in the pathobiogenesis of Alzheimer's disease.  (+info)

The native metastable fold of C1-inhibitor is stabilized by disulfide bonds. (4/83)

C1-inhibitor is a member of the serpin family of proteinase inhibitors and is an important inhibitor of complement and contact system proteinases. The native protein has the characteristic serpin feature of being in a kinetically trapped metastable state rather than in the most stable state it could adopt. A consequence of this is that it readily forms loop-sheet dimers and polymers, by a mechanism believed to be the same as observed with other serpins. An unusual feature of C1-inhibitor is that it has a unique amino-terminal domain, of unknown function, held to the serpin domain by two disulfide bonds not found in other serpins. We report here that reduction of these bonds by DTT, causes a conformational change such that the reactive center loop inserts into beta-sheet A. This form of C1-inhibitor is less stable to heat and urea than the native protein, and is more susceptible to extensive degradation by trypsin. These data show that the disulfide bonds in C1-inhibitor are required for the protein to be stabilized in the metastable state with the reactive center loop expelled from beta-sheet A.  (+info)

Hereditary angioedema with a de novo mutation of exon 8 in the C1 inhibitor gene showing recurrent edema of the hands around the peripheral joints: importance for the differential diagnosis of joint swelling. (5/83)

We describe a patient with hereditary angioedema (HAE), showing recurrent edema around the peripheral joints. Her symptoms began at the age of 18 with hand swelling distal to the wrist joints. Until she was referred to our hospital 3 years after her initial symptoms, she was still undiagnosed, although she was suspected of having rheumatoid arthritis. Laboratory examination showed reduced levels of CH50 and C4 with normal C3 levels. The C1 inhibitor (C1-INH) was decreased to 5 mg/ml, with remarkably reduced activity. Although these findings were compatible with a diagnosis of HAE, there were no episodes of skin edema in her family. To establish the diagnosis, we carried out DNA analysis of the C1-INH gene, which revealed a newly identified de novo mutation of G to A at nucleotide 16869 in exon 8. As described in this patient, localized edema around the peripheral joints may be the only manifestation of HAE. HAE should therefore be taken into consideration for the differential diagnosis of joint swelling.  (+info)

Complement components, but not complement inhibitors, are upregulated in atherosclerotic plaques. (6/83)

Complement activation occurs in atherosclerotic plaques. The capacity of arterial tissue to inhibit this activation through generation of the complement regulators C1 inhibitor, decay accelerating factor, membrane cofactor protein (CD46), C4 binding protein (C4BP), and protectin (CD59) was evaluated in pairs of aortic atherosclerotic plaques and nearby normal artery from 11 human postmortem specimens. All 22 samples produced mRNAs for each of these proteins. The ratios of plaque versus normal artery pairs was not significantly different from unity for any of these inhibitors. However, in plaques, the mRNAs for C1r and C1s, the substrates for the C1 inhibitor, were increased 2.35- and 4.96-fold, respectively, compared with normal artery; mRNA for C4, the target for C4BP, was elevated l.34-fold; and mRNAs for C7 and C8, the targets for CD59, were elevated 2.61- and 3.25-fold, respectively. By Western blotting and immunohistochemistry, fraction Bb of factor B, a marker of alternative pathway activation, was barely detectable in plaque and normal arterial tissue. These data indicate that it is primarily the classical, not the alternative pathway, that is activated in plaques and that key inhibitors are not upregulated to defend against this activation.  (+info)

A new type of acquired C1 inhibitor deficiency associated with systemic lupus erythematosus. (7/83)

Acquired C1 inhibitor (C1-INH) deficiency with consequent angioedema is a rare condition that may indicate an underlying lymphoproliferative disorder. The defect is caused by increased catabolism, which is often associated with the presence of serum autoantibodies to C1-INH. The present report describes 3 patients with systemic lupus erythematosus who developed typical symptoms of acquired angioedema, characterized by recurrent swelling of subcutaneous and mucous tissues. The 3 patients demonstrated a major classical pathway-mediated complement consumption, with very low levels of C3 antigen and decreased levels of C1-INH antigen. Neither antibodies to C1-INH nor associated lymphoproliferative disease was found. No patient had clinical and biologic signs of lupus activity at the time the angioedema occurred. All patients were treated with steroids and exhibited a good response, without relapse of angioedema and with normalization of plasma levels of C1-INH. In lupus patients who present with an angioedema syndrome, acquired or hereditary angioedema must be sought by examining parameters of the classical pathway and levels of C1-INH. Our observations suggest the existence of a new form of acquired C1-INH deficiency associated with a major classical pathway-mediated complement consumption and systemic autoimmunity.  (+info)

In vivo biosynthesis of endogenous and of human C1 inhibitor in transgenic mice: tissue distribution and colocalization of their expression. (8/83)

We have produced transgenic mice expressing human C1 inhibitor mRNA and protein under the control of the human promoter and regulatory elements. The transgene was generated using a minigene construct in which most of the human C1 inhibitor gene (C1NH) was replaced by C1 inhibitor cDNA. The construct retained the promoter region extending 1.18 kb upstream of the transcription start site, introns 1 and 2 as well as a stretch of 2.5 kb downstream of the polyadenylation site, and therefore carried all known elements involved in transcriptional regulation of the C1NH gene. Mice with high serum levels of human C1 inhibitor, resulting from multiple tandem integrations of the C1 inhibitor transgene, were selected. Immunohistochemistry in combination with in situ hybridization was applied to localize the sites of C1 inhibitor biosynthesis and to demonstrate its local production in brain, spleen, liver, heart, kidney, and lung. The distribution of human C1 inhibitor-expressing cells was qualitatively indistinguishable from that of its mouse counterpart, but expression levels of the transgene were significantly higher. In the spleen, production of C1 inhibitor was colocalized with that of a specific marker for white pulp follicular dendritic cells. This study demonstrates a stringently regulated expression of both the endogenous and the transgenic human C1 inhibitor gene and reveals local biosynthesis of C1 inhibitor at multiple sites in which the components of the macromolecular C1 complex are also produced.  (+info)

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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,
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
Patients with hereditary angioedema (HAE), suffer from recurring and mostly unforeseeable attacks of acute oedema of subcutaneous tissues of various organs. The pathophysiological correlate of this disease is a deficiency in functionally active C1-Esterase Inhibitor (C1-INH). Today, two main types of HAE are described. In HAE type I, an impaired synthesis and an elevated turnover of a normal and functional active C1-INH molecule takes place, causing reduced amounts in functionally active C1-INH. In HAE type II, normal levels of a functionally impaired C1-INH molecule are synthesized. Both defects are inherited as an autosomal dominant trait. HAE type III is limited to females and not associated with C1-INH deficiency; the pathophysiology of this type remains to be determined. Corticosteroids, antihistamines or epinephrine usually do not exert any positive effect in acute attacks caused by HAE. This is of particular importance as these types of medication are often used in case of oedema in ...
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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 ...
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:. ...
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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…
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 ...
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...
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 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
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 an episodic swelling disorder that involves the subcutaneous tissues of the extremities or the mucosa of the bowel and occasionally the tissues of the face, mouth, and pharynx or the genital area[1, 2] Attacks most commonly increase in severity for about 1.5 days and then resolve during about the same period of time. Patients are usually most bothered by the painful abdominal attacks, which can be very severe and can lead to abdominal surgery; however, the development of laryngeal angioedema can lead to asphyxiation and death. HAE types I and II are caused by mutations in 1 of the 2 gene alleles that code for the plasma protein C1 inhibitor. This protein was named for its ability to regulate the activity of the complement protein C1, but C1 inhibitor has been found to act as a regulator or inhibitor of the clotting, fibrinolytic, and kinin-generating systems in plasma as well. It is now believed that complement is not the major system responsible for HAE attacks, ...
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 ...
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 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 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
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.
Read blog posts describing how it is to have hereditary angioedema (HAE) day-by-day and offering stories and advice to help you on your journey.
We are dedicated to provide support and information on Hereditary Angioedema (HAE) to both patients and physicians, including information on recently FDA
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 ...
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 ...
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 ...
TWEETS from #ACEP17 LLSA readings reviews. 2017 LLSA review with Lovata at #ACEP17 https://t.co/dOTCNOLga5. ACEP clincial policy on aortic dissection for 1st #LLSA #ACEP17. Are there clincial decision rules for low risk pts for aortic dissection? NO #LLSA #ACEP17. Is D Dimer adequate to ID low risk aortic dissection? NO #LLSA #ACEP17. Is CTA equivalent to MRI or TEE for dx of aortic dissection? YES. #LLSA #ACEP17 level B. Can transthoracic echo rule out aortic dissection? NO #LLSA #ACEP17. When we decrease SBP and HR for aortic dissection is morbidity and mortality reduced? Not great evidence level C #LLSA #ACEP17. Angioedema up next #LLSA #ACEP17 rapid firing. Types are Histamine with and without anaphylaxis, ACE inhib and hereditary angioedema #LLSA #ACEP17. Most widely available agent for hereditary angioedema is FFP (not beat but most available) #LLSA #ACEP17. Upper airway angioedema; lower too? Direct visualization to assess. #LLSA #ACEP17. CCB overdose with literature review article. ...
...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
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…
Bowman-Birk Inhibitor Concentrate is an anticarcinogenic derived from soybeans which inhibits the protease chymotrypsin. The inhibitor was being developed at
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 ...
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 ...
Bradykinin, Angioedema, Hereditary Angioedema, Gastrointestinal Tract, Larynx, Cell, Complement, Immunity, Disease, Morbidity, Risk, Mortality, and Treatment
Wall Streets Augury: BioCryst Pharmaceuticals (Nasdaq:BCRX): Hereditary angioedema (HAE) is a very rare and potentially life-threatening genetic
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.
Here is to Lionel.. The case discussed on this Easter day relates to a ruling of the Paris Cour dappel dated January 20, 2017 which upheld a decision of the French patent and trademark office (INPI) to reject an application for a supplementary protection certificate (SPC) for a biological medicinal product.. French SPC application No. 11C0054 was filed on December 14, 2011 by Laboratoire Français du Fractionnement et des Biotechnologies (hereafter LFB), a French company specializing in medicinal products purified from human blood. The SPC application was based on French patent No. 2722992 filed on July 28, 1994, which claimed a method for the manufacture of a C1-esterase inhibitor concentrate (claim 1), as well as the concentrate obtained by the method (claim 11).. The SPC application was further based on marketing authorization (MA) No. EU/1/11/688/001 for Cinryze® comprising "C1 inhibitor, human (INN)" as active ingredient.. For those whose curiosity goes beyond patent law, C1 is one of the ...
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:. ...
Prevention of kidney transplant rejection (NCT01536379) C1 esterase inhibitor (Berinert ®) C1 inhibitor inactivates both C1r and C1s of the
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Looking for online definition of hereditary angioedema in the Medical Dictionary? hereditary angioedema explanation free. What is hereditary angioedema? Meaning of hereditary angioedema medical term. What does hereditary angioedema mean?
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 ...
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 ...
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 ...
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 ...
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.
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 ...
AB - BACKGROUND: Danazol, a drug extensively used in the management of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE), has various side effects. This study investigated the virilizing actions of this drug in 31 danazol-treated female
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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 ...
The study analyzed that HAE therapeutics pipeline comprises 20 drug candidates in different stages of development. HAE is a disease characterized by repeated incidents of swelling (angioedema). Some of the common body areas that get affected due to this disease include intestinal tract, face, limbs, and airway. The stress can cause a heart attack, but most often in the patients suffering from HAE, the swelling occurs with an unknown trigger.. Access Report Summary with Detailed TOC on "Hereditary Angioedema (HAE) Therapeutics Pipeline Analysis" at: https://www.psmarketresearch.com/market-analysis/hereditary-angioedema-therapeutics-pipeline-analysis. According to the research findings, most of the drug candidates of the HAE are being derived from the natural sources. The natural sources consist of various molecules such as gene therapies, biologicals, and RNA.. Download Sample Copy: ...
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. ...
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
Recombinant Human C1 Esterase Inhibitor/ conestat alfa].. The ongoing pediatric study is an open label Phase II clinical trial assessing safety, immunogenicity and efficacy in children 2-13 years of age with C1INH deficiency. Eight children were treated on demand for 28 HAE attacks at 50 IU/kg body weight (up to a maximum of 4200 IU). Efficacy endpoints were time to onset of relief and to minimal symptoms, assessed by the patient (assisted by their parent), using a visual analogue scale (VAS) and by physicians using an Investigator Score. Median time to beginning of relief was 60 minutes as determined by the patients and the investigators. Using the VAS, 93% of patients had onset of relief within 2 hours. No related serious adverse events, including hypersensitivity reactions, were reported.. "We believe the additional body of pediatric clinical data in children under 13 years of age are in line with the excellent data in adolescents (13-18 years of age) and adult HAE patients treated with ...
Angioedema is even caused by insect stings and pollen. Generally, there are two major types of angioedema - hereditary and acquired.
The weekly peer-reviewed general medical journal The Lancet has published data from a Phase II, double-blind, placebo-controlled, randomized clinical trial (NCT02247739) evaluating the efficacy and safety of RUCONEST (C1 esterase inhibitor [recombinant]) for the prevention of HAE attacks.. As previously reported, in a study with 32 patients RUCONEST® 50 IU/kg (max 4200 IU) demonstrated a statistically significant and clinically relevant reduction in attack frequency for both the twice-weekly and once-weekly treatment regimens when compared to placebo and was generally safe and well-tolerated in the study.. Lead-author and co-principal investigator, Marc Riedl, MD, Professor of Medicine and Clinical Director at the US HAEA Angioedema Center at The University of California San Diego, commented: "Patients with frequent HAE attacks, such as those enrolled in this study, are severely affected and have limited safe and effective choices to control their disease. The findings from this positive study ...
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.
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
Learn more about Angioedema at Colleton Medical Center DefinitionCausesRisk FactorsSymptomsDiagnosisTreatmentPreventionrevision ...
Irisin is a novel hormone like polypeptide that is cleaved and secreted by an unknown protease from fibronectin type III domain-containing protein 5 (FNDC5), a membrane- spanning protein and which is highly expressed in skeletal muscle mass, heart, adipose cells, and liver. sending the transmission to determine the function of specific cells, like skeletal muscle mass, liver, pancreas, heart, fat and the brain. The action of irisin on different targeted cells or organs in human being has exposed its physiological functions for promoting health or executing the rules of selection of metabolic illnesses. Numerous studies MLN8237 small molecule kinase inhibitor concentrate on the association of irisin with metabolic illnesses which has obtained great interest being a potential brand-new target to fight type 2 diabetes MLN8237 small molecule kinase inhibitor mellitus and insulin level of resistance. Irisin is available to boost insulin level of resistance and type 2 diabetes by raising sensitization ...
Hi, Please tell me Im not alone here. I swear my body has been falling apart the last 2 months. Let me start by saying that I had gastric sleeve surgery for weight loss in 2012. Ive been healthy s...
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. ...
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 ...
Pharming Submits Supplemental Biologics License Application to FDA for RUCONEST® for Prophylaxis of Hereditary Angioedema Attacks PR Newswire LEIDEN, The Netherlands, November 27, 2017
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 Horváth, Zsófia and Csuka, Dorottya and Vargova, Katarina and Kovács, Andrea and Lee, Sarolta and Varga, Lilian and Prohászka, Zoltán and Kiss, Róbert Gábor and Préda, István and Tóth Zsámboki, Emese (2016) Alternative complement pathway activation during invasive coronary procedures in acute myocardial infarction and stable angina pectoris. CLINICA CHIMICA ACTA, 463. pp. 138-144. ISSN ...
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 ...
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, ...
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).. ...
Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks (self or assisted administration) and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE.
From the Department of Emergency Medicine King-Drew/UCLA Medical Center, Los Angeles, California Abstract Two cases of hereditary angioedema and one of acquired angioedema are reported because of their unusual emergency department presentations. Case one is a 27-year-old man of Italian descent who visited the ED because of severe abdominal pain. He subsequently underwent an unnecessary appendectomy. Case two is a 56-year-old Caucasian man who presented to the ED because of shortness of breath, and, subsequently, he developed severe airway obstruction and was intubated. Case three is a 68-year-old black women with a history of chronic lymphocytic leukemia in remission. She was brought into the ED because of severe shortness of breath and had an emergent cricothyrotomy was performed in the ED because of upper airway obstruction and an inability to be intubated. Source Information From the Department of Emergency Medicine; King/Drew Medical Center 12021 South Wilmington Avenue; Los Angeles, ...
Angioedema is swelling in the deep layers of the skin, often seen with urticaria (hives). Angioedema most often occurs in soft tissues such as the eyelids, mouth or genitals.. Angioedema is called "acute" if the condition lasts only a short time (minutes to days). This is commonly caused by an allergic reaction to medications or foods.. Chronic recurrent angioedema is when the condition returns over a long period of time. It most often does not have an identifiable cause.. Hereditary angiodema (HAE) is a rare, but serious genetic condition involving swelling in various body parts including the hands, feet, face, intestinal wall and airways.. Learn more about skin allergies symptoms, diagnosis, treatment and management.. If you have symptoms of angioedema, call us and we can help. All of our allergists have advanced training and experience to determine what is causing your symptoms and prescribe a treatment plan to help you feel better and live better.. Source: aaaai.org. ...
This is the largest observational study of medication-related angioedema published to date. It provides important information on the risks of this condition across the spectrum of patients seen in clinics, including many patients who would not have been included in trials because of comorbidities. It is derived from a population of about 2 million VA patients prescribed antihypertensive prescriptions, focusing on a sample of nearly 600 000 patients first initiating this treatment with 833 new angioedema cases identified over a 21-month period. Furthermore, an extensive medical record review of a 14% sample indicated confirmation for over 95% of cases. From these data, we estimate an angioedema incidence in patients newly prescribed ACE of 1.97 per 1000 person years of use with relatively narrow confidence intervals (1.71 to 2.18). We project that about 1 of every 2600 new ACE users experiences angioedema within 30 days and about one of every 1000 experiences it within a year after first use. It ...
Blumenthal M, Goldberg A, Brinckmann J, eds. Herbal Medicine: Expanded Commission E Monographs. Newton, MA: Integrative Medicine Communications; 2000:84-87, 160-169, 233-239.. Chan NJ, Soliman AM. Angiotensin converting enzyme inhibitor-related angiodema: onset, presentation, and management. Ann Otol Rhino Laryngol. 2015;124(2):89-96. PMID: 25059449 www.ncbi.nlm.nih.gov/pubmed/25059449.. Chinen J, Shearer WT. Advances in basic and clinical immunology in 2006. J Allergy Clin Immunol. 2007;120(2):263-270. PMID: 17590425 www.ncbi.nlm.nih.gov/pubmed/17590425.. Cicardi M, Bergamaschini L, Cugno M, et al. Pathogenic and clinical aspects of C1 inhibitor deficiency. Immunobiol. 1998;199(2):366-376.. Ferri FF, ed. Ferris Clinical Advisor 2017. Philadelphia, PA: Elsevier; 2017.. Inomata N. Recent advances in drug-induced angioedema. Allergol Int. 2012;61(4):545-557. PMID: 23183389 www.ncbi.nlm.nih.gov/pubmed/23183389.. Johnston S, Martin LJ, Cai X. Antihistamine effect of supplemental ascorbic acid and ...
In allergic angioedema, avoidance of the allergen and use of antihistamines may prevent future attacks. Cetirizine is a commonly prescribed antihistamine for angioedema. Some patients have reported success with the combination of a nightly low dose of cetirizine to moderate the frequency and severity of attacks, followed by a much higher dose when an attack does appear. Severe angioedema cases may require desensitization to the putative allergen, as mortality can occur. Chronic cases require steroid therapy, which generally leads to a good response.
Title: "A mechanism for hereditary angioedema with normal C1 inhibitor: an inhibitory regulatory role for the factor XII heavy chain". Mentor: Dr. James Morrissey. ...
1. Stanozolol has been used in both animal and human patients for a number of conditions. In humans, it has been demonstrated to be successful in treating anaemia and hereditary angioedema. Veterinarians may prescribe the drug to improve muscle growth, red blood cell production, increase bone density and stimulate the appetite of debilitated or weakened animals.. 2. Stanozolol is one of the anabolic steroids commonly used as performance enhancing drugs and is banned from use in sports competition under the auspices of the International Association of Athletics Federations (IAAF) and many other sporting bodies. Additionally, stanozolol has been used in US horse racing. ...
Since 2010, BioRx has become a prominent player in the Hereditary Angioedema space and a major player in the Alpha-1 antitrypsin deficiencies space.. "Some of the other changes since 2010 are we announced that we were going to be a semi-exclusive distribution partner for a firm out of New Jersey called NPS Pharmaceuticals and we opened three new regional pharmacy and distribution centers," Hill says. "Those are in Boston, Scottsdale, Ariz., and San Diego, Calif. Those are three large investments for us.". Needless to say BioRx has been doing the right things to remain on a growth track. Now Rielly and Hill have to keep it going.. Heres how they have grown the company through strategic planning and developing the right partnerships.. Take advantage of growth drivers. When Rielly and Hill first started BioRx, they had a different idea behind specialty pharmaceuticals than most other national companies. While others were switching to a less personalized mail order model, Rielly and Hill saw an ...
Angioedema and bupropion permanent swelling treatment - Fast order processing. Visa&MasterCard payment cards. We deliver orders to 135 Countries. We accept Bitcoin. We work 20 years.
Detailed information about hives & angioedema (urticaria), red welts and swellings, their symptoms, causes, treatments, prevention and skin care.
Learn about causes of hives (allergy, stress), rash symptoms (skin welts, raised red itchy bumps), and see pictures. Hives treatment aims to alleviate symptoms. Dermatographism and swelling (angioedema) may accompany hives (urticaria).
Are there any other precautions or warnings for this medication?. Before you begin using a medication, be sure to inform your doctor of any medical conditions or allergies you may have, any medications you are taking, whether you are pregnant or breast-feeding, and any other significant facts about your health. These factors may affect how you should use this medication.. Angioedema: Ramipril may cause a serious allergic reaction called angioedema, which may be fatal if not treated promptly. If you have difficulty breathing or notice hives or swelling of the face, lips, tongue, or throat, stop taking this medication and get emergency medical help at once. Other ACE inhibitors should not be taken in the future. People who have had angioedema caused by other substances may be at increased risk of angioedema while taking this medication.. Blood disorders: In rare cases, a low white blood cell count has been reported with people taking this medication. Your doctor may occasionally monitor your level ...
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. ...
PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of Health (NIH) free digital archive of biomedical and life sciences journal literature and is a member of the broader PMC International (PMCI) network of e-repositories.
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 ...
Purchase Angioedema, An Issue of Immunology and Allergy Clinics of North America, Volume 37-3 - 1st Edition. Print Book & E-Book. ISBN 9780323532372, 9780323532389
Allergy Pathogenesis Hypersensitivity Reactions, Delayed Hypersensitivity Reactions, Immediate Indoor Aeroallergens Asthma Allergic and Environmental Asthma Vocal Cord Dysfunction Complement-Related Abnormalities Complement Deficiencies Hereditary Angioedema Hypocomplementemia Immunodeficiencies DiGeorge Syndrome Hypogammaglobulinemia Immunoglobulin A Deficiency Immunoglobulin D Deficiency Immunoglobulin G Deficiency Immunoglobulin M Deficiency Panhypogammaglobulinemia Reticular Dysgenesis Severe Combined Immunodeficiency Wiskott-Aldrich Syndrome Major Allergic…
Complement proteins C1-inhibitor C3-convertase Factor VIII Factor XIII Protein C Protein S Protein Z Protein Z-related protease ... A list of proteins (and protein complexes). This list aims to organize information on the protein universe. All proteins can be ... G-protein-coupled receptor Rhodopsin Estrogen receptor Histones Protamines CI protein % Transcription regulatory proteins that ... For more information about categorizing protein types, see List of types of proteins. Arp2/3 Coronin Dystrophin FtsZ Keratin ...
... is not essential for C1-inhibitor to inhibit proteases. This domain has no similarity to other proteins. C1-inhibitor is highly ... This way, C1-inhibitor prevents the proteolytic cleavage of later complement components C4 and C2 by C1 and MBL. Although named ... C1-inhibitor (C1-inh, C1 esterase inhibitor) is a protease inhibitor belonging to the serpin superfamily. Its main function is ... Note that C1-inhibitor is the most important physiological inhibitor of plasma kallikrein, fXIa, and fXIIa. C1-inhibitor is the ...
... breakdown of fibrin clots plasminogen Inhibitors of fibrinolysis α2-antiplasmin Complement components C1-9, Complement ... The liver plays the major role in producing proteins that are secreted into the blood, including major plasma proteins, factors ... Vitamin D-binding protein, carries vitamin D Insulin-like growth factor 1, a polypeptide protein hormone which plays an ... forming a blood clot that stops bleeding C-reactive protein, opsonin on microbes, acute phase protein Various other globulins ...
Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, ... C1-inhibitor deficiency or hereditary angioedema will have low C4 with normal C1 levels. Acquired hypocomplementemia may occur ... Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. ... Davis, Alvin E.; Mejia, Pedro; Lu, Fengxin (1 October 2008). "BIOLOGICAL ACTIVITIES OF C1 INHIBITOR". Molecular immunology. 45 ...
C1 inhibitor (C1-INH) antigenic protein, C1 inhibitor (C1-INH) functional level if available.Analysis of complement C1 ... Other treatment modalities can stimulate the synthesis of C1 inhibitor, or reduce C1 inhibitor consumption. Purified C1 ... In this type, atypical C1-inhibitor proteins are produced which are less capable of suppressing activation of the complement ... There are three types of C1 inhibitor deficiency: HAE type I is primarily caused by a deficiency in blood proteins (C1 esterase ...
The classical pathway is inhibited by C1-inhibitor, which binds to C1 to prevent its activation.[citation needed] ... Over 30 proteins and protein fragments make up the complement system, including serum proteins, and cell membrane receptors. ... complement factor B, and complement factor I, as well as deletion of complement factor H-related 3 and complement factor H- ... Deficiencies in complement regulatorsEdit. Mutations in the complement regulators factor H and membrane cofactor protein have ...
Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... C1-inhibitor deficiency (hereditary angioedema) Factor I deficiency (pyogenic infections) Factor H deficiency (haemolytic- ... The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that ... MASP2 deficiency Complement receptor 3 (CR3) deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex ...
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 ... Most of the complement control proteins act on the convertases, C3b.Bb and C4b.2a, which are bimolecular complexes formed early ...
Among soluble inhibitors there are factor H, C1 inhibitor, C4b-binding protein, factor I, S protein or clusterin, the membrane- ... Non-apoptotic cells also express complement inhibitors, preventing the assembly of C3 convertase or the lytic pore. ... bound inhibitors are CR1, membrane cofactor protein (MCF), decay accelerating factor (DAF) or protectin (CD59). Phagocytes are ... Besides complement particles C1q and C3b which help to opsonize the apoptotic cells, also thrombospondin, pentraxins (C- ...
The classical pathway is inhibited by C1-inhibitor, which binds to C1 to prevent its activation.[citation needed] C3-convertase ... Over 30 proteins and protein fragments make up the complement system, including serum proteins, and cell membrane receptors. ... Polymorphisms of complement component 3, complement factor B, and complement factor I, as well as deletion of complement factor ... The complement system is regulated by complement control proteins, which are present at a higher concentration in the blood ...
... which result in either diminished levels of the C1-inhibitor protein (type I HAE) or dysfunctional forms of the same protein ( ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... Ruconest (C1-inhibitor). References[edit]. *^ a b c d e f g h i j k l m n o p q r s t Bernstein, JA; Cremonesi, P; Hoffmann, TK ... Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered ...
... (EC 3.4.21.42, C1 esterase, activated complement C1s, complement C overbar 1r, C1s) is a protein ... Katz Y, Strunk RC (March 1989). "Synthesis and regulation of C1 inhibitor in human skin fibroblasts". Journal of Immunology. ... complement activation, lectin pathway. • complement activation. • regulation of complement activation. Sources:Amigo / QuickGO ... protein binding. • hydrolase activity. • metal ion binding. • identical protein binding. • serine-type endopeptidase activity. ...
C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity. C1- ... The classical complement pathway can be initiated by the binding of antigen-antibody complexes to the C1q protein. The globular ... Lack of regulation of the classical complement pathway through the deficiency in C1-inhibitor results in episodic angioedema. ... Alternative complement pathway - another complement system pathway Lectin pathway - another complement system pathway Noris, ...
C1-inhibitor (which protects the body from excessive protease-triggered activation of its own complement system), antithrombin ... Natural protease inhibitors include the family of lipocalin proteins, which play a role in cell regulation and differentiation ... Other natural protease inhibitors are used as defense mechanisms. Common examples are the trypsin inhibitors found in the seeds ... The natural protease inhibitors are not to be confused with the protease inhibitors used in antiretroviral therapy. Some ...
1994). "Regulation of the synthesis of C1 subcomponents and C1-inhibitor". Behring Inst. Mitt. (93): 196-203. PMID 8172568. ... Complement C1q subcomponent subunit A is a protein that in humans is encoded by the C1QA gene. This gene encodes a major ... 1994). "The envelope glycoprotein of HIV-1 gp120 and human complement protein C1q bind to the same peptides derived from three ... 1994). "Expression of the components and regulatory proteins of the classical pathway of complement in normal and diseased ...
... which result in either diminished levels of the C1-inhibitor protein (type I HAE) or dysfunctional forms of the same protein ( ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... Acute treatment consists of C1-INH (C1-esterase inhibitor) concentrate from donor blood, which must be administered ... C1-esterase (aka: C1-inhibitor or C1INH), and continuous production of kallikrein, another process inhibited by C1INH. This ...
... on chromosome 6 at 6p21.3 Polymorphisms in genes for complement system proteins: The genes for the complement system proteins ... Six mutations of the gene SERPING1 (Serpin Peptidase Inhibitor, Clade G (C1 Inhibitor), Member 1) are associated with AMD. ... Complement factor H (CFH) is an important inhibitor of this inflammatory cascade, and a disease-associated polymorphism in the ... Rohrer B, Long Q, Coughlin B, Renner B, Huang Y, Kunchithapautham K (2010). "A targeted inhibitor of the complement alternative ...
... complement c1 inactivator proteins MeSH D12.776.124.486.274.920.250.500 -- complement c1 inhibitor protein MeSH D12.776.124.486 ... complement c5a, des-arginine MeSH D12.776.124.486.274.050 -- complement c1 MeSH D12.776.124.486.274.050.270 -- complement c1q ... complement c3b inactivator proteins MeSH D12.776.124.486.274.920.325.200 -- complement factor h MeSH D12.776.124.486.274.920. ... 325.210 -- complement factor i MeSH D12.776.124.486.274.920.662 -- complement c4b-binding protein MeSH D12.776.124.486.274.930 ...
... that are not involved in coagulation such as trypsin and the C1s subunit of the enzyme C1 involved in the classical complement ... Antithrombin is a serpin (serine protease inhibitor) and is thus similar in structure to most other plasma protease inhibitors ... As deduced from protein and cDNA sequencing, cow, sheep, rabbit and mouse antithrombins are all 433 amino acids in length, ... Unexpectedly the protein crystallized as a heterodimer composed of one molecule of native antithrombin and one molecule of ...
C1-inhibitor (which protects the body from excessive protease-triggered activation of its own complement system), antithrombin ... Natural protease inhibitors include the family of lipocalin proteins, which play a role in cell regulation and differentiation ... Inhibitors[edit]. Main articles: Protease inhibitor (biology) and Protease inhibitor (pharmacology). The activity of proteases ... Thus, protease inhibitors are developed as antiviral means. Other natural protease inhibitors are used as defense mechanisms. ...
... soluble complement receptor type 1, anti-C5 antibodies, or C1 inhibitor (C1-INH). Disadvantages of this approach include the ... Hormone and protein differences - Some proteins will be molecularly incompatible, which could cause malfunction of important ... The binding of XNAs initiate complement activation through the classical complement pathway. Complement activation causes a ... Interruption of the complement cascade The recipient's complement cascade can be inhibited through the use of cobra venom ...
... s are classed as irreversible inhibitors and as suicide inhibitors since each serpin protein permanently inactivates a ... Beinrohr L, Harmat V, Dobó J, Lörincz Z, Gál P, Závodszky P (July 2007). "C1 inhibitor serpin domain structure reveals the ... Mollnes TE, Jokiranta TS, Truedsson L, Nilsson B, Rodriguez de Cordoba S, Kirschfink M (September 2007). "Complement analysis ... it became clear that these inhibitors were part of superfamily of related proteins that included both protease inhibitors (e.g ...
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... Genetic disorder, protein biosynthesis: Transcription factor/coregulator deficiencies. (1) Basic domains. 1.2. *Feingold ...
"Fluid-phase interaction of C1 inhibitor (C1 Inh) and the subcomponents C1r and C1s of the first component of complement, C1". ... Complement C1r subcomponent (EC 3.4.21.41, activated complement C1r, C overbar 1r esterase, C1r) is a protein involved in the ... C1r along with C1q and C1s form the C1 complex, which is the first component of the serum complement system. C1r is an enzyme ... Arlaud GJ, Gagnon J (April 1983). "Complete amino acid sequence of the catalytic chain of human complement subcomponent C1-r". ...
Complement pathway inhibitors C1-inhibitor - Classical, Lectin, Alternate Decay-accelerating factor (CD59) - Classical, Lectin ... see complement proteins section) Collectins Mannan-binding lectin (MBL) Surfactant protein A (SP-A) Surfactant protein D (SP-D ... system Complement system Classical complement pathway Mannan-binding lectin pathway Alternate complement pathway Complement ... CL-L1 CL-P1 CL-K1 Peptidoglycan recognition proteins (PGRs) PGLYRP1 PGLYRP2 PGLYRP3 PGLYRP4 Ficolins FCN1 FCN2 FCN3 Complement ...
Inhibitors. *CLA: C1-inhibitor. *Decay-accelerating factor/CD59. *Factor I. *CL: C4BP ... MAC is composed of a complex of four complement proteins (C5b, C6, C7, and C8) that bind to the outer surface of the plasma ... The membrane attack complex is initiated when the complement protein C5 convertase cleaves C5 into C5a and C5b. All three ... Stanley KK, Marazziti D, Eggertsen G, Fey GH (1988). "Relationships between the gene and protein structure in human complement ...
Complement. deficiency. *C1-inhibitor (Angioedema/Hereditary angioedema). *Complement 2 deficiency/Complement 4 deficiency ... Next, the NK cells which have Fc Receptors will bind to that antibody, inducing the NK cell to release proteins such as ... ADCC is independent of the immune complement system that also lyses targets but does not require any other cell. ADCC requires ... During replication of a virus some of the viral proteins are expressed on the cell surface membrane of the infected cell. ...
The complement system includes blood proteins that can cause cell death after an antibody binds to the cell surface (the ... Complement-dependent cytotoxicity occurs when antibodies bind to the cancer cell surface, the C1 complex binds to these ... Antibody BGB-A317 is a PD-1 inhibitor (designed to not bind Fc gamma receptor I) in early clinical trials. In May 2016, PD-L1 ... Complement can lead to cell death by activation of the membrane attack complex, known as complement-dependent cytotoxicity; ...
... have serum levels of functionally active inhibitor of the first component of complement (C1 INH) between 5 and 30% of normal, ... the functional protein comprised 43% of the total. Thus, type II HANE cells synthesized functional C1 INH at a much greater ... amounts of steady-state C1 INH mRNA levels paralleled rates of C1 INH synthesis, indicating that control of C1 INH synthesis ... Synthesis of C1 inhibitor in fibroblasts from patients with type I and type II hereditary angioneurotic edema.. ...
Complement, Immunity, Disease, Morbidity, Risk, Mortality, and Treatment ... Human complement regulators C4b-binding protein and C1 esterase inhibitor interact with a novel outer surface protein of ... Human pasteurized C1-inhibitor concentrate for the treatment of hereditary angioedema due to C1-inhibitor deficiency. Abstract ... Treatment with C1-inhibitor concentrate does not induce IgM type anti-C1 inhibitor antibodies in patients with hereditary ...
C1 Protein Inhibitor) Determination Reagents" , "Reagents, Immunoassay, Protein, Complement Component, C1 Inhibitor" ... an esterase inhibitor of the C1 protein found in the classic pathway of the complement components proteins. The absence of this ... IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C1 Inhibitor. Definition : Immunoassay reagents intended to ... Home > Specialties > IVD Test Reagent/Kits, Immunoassay, Protein, Complement Component, C1 Inhibitor ...
Complement C1 Inhibitor Protein. Complement Inactivating Agents. Immunosuppressive Agents. Immunologic Factors. Physiological ... markedly decreased C1 inhibitor activity, decreased level of C1 inhibitor antigen and a decreased level of C4. ... The changes in the manufacturing process of C1-esteraseremmer-N, compared to Cetor® (the currently marketed C1-inhibitor ... The changes in the manufacturing process of C1-esteraseremmer-N, compared to Cetor® (the currently marketed C1-inhibitor ...
Complement C1 Inhibitor Protein. Complement Inactivating Agents. Immunosuppressive Agents. Immunologic Factors. Physiological ... Blom AM, Villoutreix BO, Dahlbäck B. Complement inhibitor C4b-binding protein-friend or foe in the innate immune system? Mol ... Davis AE 3rd, Lu F, Mejia P. C1 inhibitor, a multi-functional serine protease inhibitor. Thromb Haemost. 2010 Nov;104(5):886-93 ... The specific aim of this study is to evaluate the effect of recombinant human C1-inhibitor (rhC1INH), as a kidney recipient ...
Complement C1 Inhibitor Protein. Complement Inactivating Agents. Immunosuppressive Agents. Immunologic Factors. Physiological ... Experimental: Recombinant Human C1 Inhibitor Drug: rhC1INH Patients up to 84 kg will receive one i.v. injection of Ruconest at ... Pharmacokinetics and Efficacy of Recombinant Human C1 Inhibitor for the Treatment of Acute Attacks in Pediatric Patients With ...
Complement C1 Inhibitor Protein. Complement Inactivating Agents. Immunosuppressive Agents. Immunologic Factors. Physiological ... C1 Inhibitor (C1INH) and C4 Levels [ Time Frame: 18 days in each treatment period ]. *Number of Subjects With C1INH Antibodies ... Drug Information available for: SERPING1 protein, human Genetic and Rare Diseases Information Center resources: Hereditary ... C1 esterase inhibitor (human). Experimental: IV CINRYZE First, Then SC CINRYZE Dose 2 Biological: CINRYZE C1 esterase inhibitor ...
... complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite ... complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite ... complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite ... complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite ...
... 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 has long been regarded as a pivotal effector arm of the innate immune response, eliciting important immunoregulatory ... Human Astrovirus Coat Protein: A Novel C1 Inhibitor Neel K. Krishna, Kenji M. Cunnion ... protein structures, design of complement inhibitors, and complement assays discussed during the conference. ... Role of Complement in Motor Neuron Disease: Animal Models and Therapeutic Potential of Complement Inhibitors ...
2010) Human complement regulators C4b-binding protein and C1 esterase inhibitor interact with a novel outer surface protein of ... these proteins are the surface lipoproteins vlp and vsp, which are also known to be its main proinflammatory proteins (30). The ... 2002) Characterisation of silent and active genes for a variable large protein of Borrelia recurrentis. BMC Infect Dis 2:25. ... 3). Strikingly, these genes mark the end of mapping for plasmid pl33 and pl37, while pl53 retains a hypothetical protein at its ...
... zinc finger protein 23 (ZNF23), collagen type XXVII al (COL27AI), Kazrin isoform-1, keratin-associated protein 10-9 (KRTAPIO-9 ... Huntingtin (HTT), microtubule associated protein 9 (MAP9), coiled-coil domain-containing protein 13 (CCDC13), inositol ... ALLI-fused gene from chromosome 4 protein (AR4)/Fragile X Mental Retardation 2 (FMR2) family member 3 (AFF3), transthyretin ( ... methods include providing a sample from the subject and detecting the level of one or more of growth arrest-specific protein 1 ...
Complement C1 Esterase inhibitor. 15-35. Complement C1r Component. -. Complement C4 Binding Protein. -. ... Many of these proteins are produced on a large-scale custom basis under GMP for our manufacturing customers. For more extensive ... It is estimated that plasma may contain as many as 40,000 different proteins from about 500 gene products. Sigma-Aldrich has ... From "The Plasma Proteins", Volume IV, 2nd Edition, 1984, Frank W. Putnam. ...
Complement C1 Inactivator Proteins/genetics. Complement C1 Inhibitor Protein/chemistry. Complement C1 Inhibitor Protein/ ... 0 (Angiotensin-Converting Enzyme Inhibitors); 0 (Complement C1 Inactivator Proteins); 0 (Complement C1 Inhibitor Protein); 0 ( ... 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 (Peptides); 0 (Recombinant Proteins); 0 (SERPING1 ...
Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) ... human SERPING1 protein * Complement C1 Inhibitor Protein * Hereditary Angioedemas * ecallantide * Bradykinin Receptor ...
... exp Complement C1 Inactivator Proteins/ OR exp Complement C1 Inhibitor Protein/ OR exp Complement C1/ OR c1 esterase inhibitor. ... 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 ...
C1-INH,May,Aid,in,Prevention,of,Antibody-Mediated,Rejection,Following,Kidney,Transplant,biological,advanced biology technology, ... SAN FRANCISCO July 30 2014 /-...The study shows that post-transplant treatment with C1-INH results in ... Antibody-mediated ... C1-INH is a human protein and an important inhibitor of the complement system. ... C1-INH function and antigen levels in blood increased with C1-INH treatment [C1 function (p=0.0007) and C1-INH antigen percent ...
C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis ... C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis ... C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis ... C1 INH) deficiency. The preparation of nanofiltered C1 INH (C1 INH-nf) used in this study is indicated for routine prophylaxis ...
... complement C4-B (231%), serum amyloid A-4 protein (210%), inter-alpha-trypsin inhibitor heavy chain H4 (191%), and alpha-1- ... complement C4-B (231%), serum amyloid A-4 protein (210%), inter-alpha-trypsin inhibitor heavy chain H4 (191%), and alpha-1- ... Proteomic analysis indicated large increases for immune-related proteins involved with complement activation and the acute ... The blood proteins with the largest increase were complement component C7 (359%), ...
C1 inhibitor is one of the proteins in the complement system, which is part of the immune system. Symptoms usually start during ... acquired C1 inhibitor deficiency) are caused by a deficiency or malfunction of C1 inhibitor, which is part of the immune system ... Acquired C1 Inhibitor Deficiency). By Peter J. Delves, PhD, Professor of Immunology, Division of Infection & Immunity, Faculty ... These drugs, taken by mouth, can stimulate the body to produce more C1 inhibitor, but they may be less effective for acquired ...
Complement 1 inhibitor (C1-INH)* is a critically important protein that controls activation of multiple plasma mediator ... We studied complement 1 inhibitor (C1-INH) as an inhibitor of the alternative complement pathway. C1-INH prevented lysis, ... C1-INH and alternative-complement activities in C1-INH depleted serum. C1-INH functional activity (white bars) and alternative- ... All the above attempts failed to detect interaction between labeled proteins and C1-INH or control proteins. However, C1-INH ...
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- ... esterase inhibitor (Cinryze). 7. Ublituximab for Acute Neuromyelitis Optica (NMO) Relapses. Active, not recruiting. NCT02276963 ...
... of C1 inhibitor (C1-INH) - a protein that prevents complement activation by both the classic route and the mannan-binding ... Fresh-frozen plasma contains several complement proteins and is considered first-line therapy where C1 esterase inhibitor ... C1 esterase inhibitor protein can be used for long-term prophylaxis in selected patients.3 ... The most effective medical treatment for acute episodes of HA is intravenous C1 esterase inhibitor protein,3 which is ...
... analytes include C3 complement, C4 complement, C1 inhibitor, C-reactive protein, and/or rheumatoid fator); polyclonal/ ... serum proteins, cell surface proteins, protein fragments, modified proteins), any infectious disease (e.g., bacterial based on ... a viral protein (e.g., hemagglutinin), an extracellular matrix protein, a lectin, or an ectodomain of a cell surface protein. ... The binding moiety can also be an antibody directed toward an antigen or any protein-protein interaction. Also, the binding ...
Complement C1 Inhibitor Protein Bordetella pertussis Complement C3b Complement Membrane Attack Complex ... Bordetella pertussis isolates vary in their interactions with human complement components article. Brookes, C., Freire-Martin, ...
  • In both type I and II HANE cells, amounts of steady-state C1 INH mRNA levels paralleled rates of C1 INH synthesis, indicating that control of C1 INH synthesis occurred at pretranslational levels. (jci.org)
  • These data suggest that the lower than expected amounts of functionally active C1 INH in type I HANE may be due, in part, to a decrease in rate of synthesis of the protein, and that the expressions of the normal C1 INH allele in HANE is influenced by the type of abnormal allele present. (jci.org)
  • 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. (jci.org)
  • We studied the synthesis of C1 INH in skin fibroblast lines, which produce easily detectable amounts of C1 INH. (jci.org)
  • In type I HANE cells, C1 INH synthesis was 19.6 +/- 4.0% (mean +/- SD) of normal, much less than the 50% predicted. (jci.org)
  • Objectives: To determine serum concentrations of proinflammatory (C reactive protein, complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite cyclists before and during a three week cycle tour. (edu.au)
  • abstract = "Objectives: To determine serum concentrations of proinflammatory (C reactive protein, complement C3 and C4) and anti-inflammatory (α1 antitrypsin, C1 esterase inhibitor (C1-INH)) acute phase proteins in elite cyclists before and during a three week cycle tour.Methods: Seventeen professional cyclists participating in the Vuelta a Espańa volunteered for the study. (edu.au)
  • C1-INH prevented lysis, induced by the alternative complement pathway, of paroxysmal nocturnal hemoglobinuria (PNH) erythrocytes in human serum. (rupress.org)
  • Removal of C1-INH from serum, in the presence of Mg-EGTA with an anti-C1-INH immunoabsorbant, markedly increased alternative-pathway lysis. (rupress.org)
  • Arp2/3 Coronin Dystrophin FtsZ Keratin Myosin Tubulin Collagen Elastin F-spondin Pikachurin Fibronectin Serum Amyloid P Component Serum albumin Complement proteins C1-inhibitor C3-convertase Factor VIII Factor XIII Protein C Protein S Protein Z Protein Z-related protease inhibitor Thrombin Von Willebrand Factor C-reactive protein Hemoglobin (oxyhemoglobin and deoxyhemoglobin) Cadherin Ependymin Integrin NCAM Selectin Ion pumping enzymes are in the enzymes section. (wikipedia.org)
  • Resistance to complement mediated killing, or serum resistance, is a common trait of pathogenic bacteria. (semanticscholar.org)
  • Surrogate markers of kidney injury including serum creatinine and cystatin C and urinary Neutrophil gelatinase-associated lipocalin and TIMP2 * Insulin-like growth factor-binding protein 7 (IGFBP7) will be assessed over a 48 hours time period. (centerwatch.com)
  • Serum C1 esterase inhibitor levels immediately before and 10 minutes after administration of Conestat alfa or placebo will be assessed. (centerwatch.com)
  • HAE is diagnosed by the patient's appearance, family history, blood testing for serum C4 levels, and other complement levels such as C1, C2 and C4. (medicinenet.com)
  • The transfer of the major egg yolk proteins such as Very Low Density Lipoproteins (VLDL) containing essentially apovitellenin and apolipoprotein-B, but also vitellogenins and some other plasma proteins from the blood such as serum albumin to the interstitial fluid of the thecae is possible due to the presence of broad discontinuities in the capillary endothelium. (biomedcentral.com)
  • Two types exist: type I, in which reduced serum levels of functionally active C1 inactivator occur, and type II, in which normal or even elevated levels of functionally inactive C1 inactivator are present. (viracor-eurofins.com)
  • Serum sickness is an example of immune complex clearance by complement proteins. (lecturio.com)
  • Over 30 proteins and protein fragments make up the complement system, including serum proteins , and cell membrane receptors . (wikipedia.org)
  • Laboratory investigations showed the following: The complete blood count (CBC), liver function test (LFT), serum urea, creatinine, electrolytes (U/E/Cr), ESR and C- reactive protein (CRP) were all unremarkable. (omjournal.org)
  • Among the investigated isolates, growth of ZWU3 Ny3 was not affected while growth of VS116 and Bv9 was strongly inhibited in the presence of 50% human serum.In contrast, no surface-deposited components and no aberrations in cell morphology were detected for serum-resistant ZWU3 Ny3.Taken together, these findings suggest that certain B. valaisiana isolates differ in their capability to resist complement-mediating killing by human serum. (nih.gov)
  • Spirochetes belonging to the Borrelia (B.) burgdorferi sensu lato complex differ in their resistance to complement-mediated killing, particularly in regard to human serum. (nih.gov)
  • In the present study, we elucidate the serum and complement susceptibility of B. valaisiana, a genospecies with the potential to cause Lyme disease in Europe as well as in Asia. (nih.gov)
  • Taken together, these findings suggest that certain B. valaisiana isolates differ in their capability to resist complement-mediating killing by human serum. (nih.gov)
  • The molecular mechanism utilized by B. valaisiana to inhibit bacteriolysis appears not to involve binding of the key host complement regulators of the alternative, classical, and lectin pathways as already known for serum-resistant Lyme disease or relapsing fever borreliae. (nih.gov)
  • We began these experiments with the hypothesis that C1-INH regulates factor D activity. (rupress.org)
  • G-protein-coupled receptor Rhodopsin Estrogen receptor Histones Protamines CI protein % Transcription regulatory proteins that are receptors are in the receptors section. (wikipedia.org)
  • In analogy to the newly described neuroimmune regulatory proteins also known as "don't eat me" signals (CD200, CD47, CD22, fractalkine, semaphorins), we herein identify the key role of complement regulator factor H (fH) in controlling neuroinflammation initiated in an acute mouse model of Ab-dependent experimental autoimmune encephalomyelitis. (jimmunol.org)
  • Membrane complement regulatory proteins (mCRPs) such as CD46, CD55, and CD59, protect host cells from complement attack. (arvojournals.org)
  • They had a familial history of angioedema and normal C1 inhibitor (C1-INH) levels, leading to the diagnosis of HAE with normal C1-INH (HAEnC1-INH) or HAE type III. (bireme.br)
  • Patients treated with C1-INH experienced increased C3 levels on day 30 (p=0.005), while C4 levels were significantly higher at all time points. (bio-medicine.org)
  • Doctors diagnose hereditary or acquired angioedema by measuring C1 inhibitor levels or activity in a sample of blood. (merckmanuals.com)
  • Flavivirus nonstructural protein 1 (NS1) is a secreted nonstructural glycoprotein that accumulates in plasma to high levels and is displayed on the surface of infected cells but absent from viral particles. (jimmunol.org)
  • Mouse neurons expressed other complement regulators crry and low levels of CD55. (jimmunol.org)
  • CD59 protein levels in cultured hRPE cells were higher than in native hRPE cells. (arvojournals.org)
  • Normal levels during symptomatic periods rule out the diagnosis, whereas decreased levels warrant determination of C1 esterase inhibitor titer by immunoassay or functional assay. (utmb.edu)
  • Levels of proteins in ischemic brain were measured by immunofluorescence and western blotting. (springer.com)
  • There is an additional type in which C1 levels are normal but C1 function is decreased (type II HAE). (bionity.com)
  • In 85% of the cases, the levels of C1-inhibitor are low, while in 15% the protein circulates in normal amounts but it is dysfunctional. (wikipedia.org)
  • Membrane proteins, CD46 and CD55, also control C3 levels, and defects in CD46 have also been associated with atypical hemolytic uremic syndrome and macular degeneration. (psychiatryadvisor.com)
  • Whereas uEVs of young patients with ADPKD and preserved kidney function already had higher levels of complement, only uEVs of patients with advanced stages of ADPKD had increased levels of villin-1, periplakin, and envoplakin. (asnjournals.org)
  • 7 December 2011 - US drug discovery and development company ISIS Pharmaceuticals Inc (NASDAQ:ISIS) reported on Tuesday positive data from a Phase I clinical trial with ISIS-APOCIIIRx, demonstrating that ISIS-APOCIIIRx treatment led to fast, dose-dependent reductions of up to 78% in apolipoprotein C-III (apoC-III) protein and up to 44% in blood triglyceride levels. (thefreedictionary.com)
  • Blood tests that measure activity or levels of C1 inhibitor, confirm diagnosis. (unitedhealthdirectory.com)
  • C1 inhibitor maintains the natural regulation of the contact, complement, and fibrinolytic systems, that when left unregulated, can initiate or perpetuate an attack by consuming the already low levels of endogenous C1 inhibitor in HAE patients. (bio-medicine.org)
  • however, his plasma complement levels for C3 was low at 74 mg/dL (N: 90-180) and C4 was normal. (omjournal.org)
  • Edema formation is related to reduction or dysfunction of C1 inhibitor, and conventional therapy with antihistamines and corticosteroids is ineffective. (nih.gov)
  • Type 2 accounts for 15% to 20% of HAE and is caused by C1 dysfunction. (marketresearchreports.biz)
  • Ehrlich believed that each antigen-specific amboceptor has its own specific complement, whereas Bordet believed that there is only one type of complement. (wikipedia.org)
  • C1-INH interacts with C3b to inhibit binding of factor B to C3b. (rupress.org)
  • In addition, C1-INH has been reported to remove the intact C1qrs complex from an activating surface ( 4 ) and to inhibit autoactivation of C1 ( 5 ). (rupress.org)
  • The complement cascade plays a central role in the modulation of inflammatory responses. (arvojournals.org)
  • When someone has a heart attack, for instance, the lack of oxygen in heart cells causes necrosis in heart cells: Dying heart cells spill their contents in the extracellular environment, which triggers the complement cascade. (wikipedia.org)
  • In part A, the pharmacokinetics of C1-esteraseremmer-N in patients with hereditary angioedema will be compared with the current registered product, Cetor®, in a randomised, blinded cross-over design. (clinicaltrials.gov)
  • Vitronectin binds to the head region of Moraxella catarrhalis ubiquitous surface protein A2 and confers complement-inhibitory activity. (semanticscholar.org)
  • This powerful surveillance system comprises a network of precursors, regulatory and inhibitory proteins that can be immediately activated upon recognition of invading microorganisms ( 9 , 10 ). (frontiersin.org)