Complement c1 inhibitor protein. Medical search

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

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

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

The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. C3a, a 77-amino acid peptide, is a mediator of local inflammatory process. It induces smooth MUSCLE CONTRACTION, and HISTAMINE RELEASE from MAST CELLS and LEUKOCYTES. C3a is considered an anaphylatoxin along with COMPLEMENT C4A; COMPLEMENT C5A; and COMPLEMENT C5A, DES-ARGININE.

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

The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. C5a is a 74-amino-acid glycopeptide with a carboxy-terminal ARGININE that is crucial for its spasmogenic activity. Of all the complement-derived anaphylatoxins, C5a is the most potent in mediating immediate hypersensitivity (HYPERSENSITIVITY, IMMEDIATE), smooth MUSCLE CONTRACTION; HISTAMINE RELEASE; and migration of LEUKOCYTES to site of INFLAMMATION.

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

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

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

The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.

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

A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. It is a polypeptide chain cross-linked by 32 disulfide bonds. C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. It is encoded by gene C6.

A 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.

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

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

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

A 63-kDa serum glycoprotein encoded by gene C9. Monomeric C9 (mC9) binds the C5b-8 complex to form C5b-9 which catalyzes the polymerization of C9 forming C5b-p9 (MEMBRANE ATTACK COMPLEX) and transmembrane channels leading to lysis of the target cell. Patients with C9 deficiency suffer from recurrent bacterial infections.

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

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

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

A 80-kDa subcomponent of complement C1, existing as a SERINE PROTEASE proenzyme in the intact complement C1 complex. When COMPLEMENT C1Q is bound to antibodies, the changed tertiary structure causes autolytic activation of complement C1r which is cleaved into two chains, A (heavy) and B (light, the serine protease), connected by disulfide bonds. The activated C1r serine protease, in turn, activates COMPLEMENT C1S proenzyme by cleaving the Arg426-Ile427 bond. No fragment is released when either C1r or C1s is cleaved.

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

A 93-kDa serum glycoprotein encoded by C7 gene. It is a polypeptide chain with 28 disulfide bridges. In the formation of MEMBRANE ATTACK COMPLEX; C7 is the next component to bind the C5b-6 complex forming a trimolecular complex C5b-7 which is lipophilic, resembles an integral membrane protein, and serves as an anchor for the late complement components, C8 and C9.

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

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

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

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

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

A 150-kDa serum glycoprotein composed of three subunits with each encoded by a different gene (C8A; C8B; and C8G). This heterotrimer contains a disulfide-linked C8alpha-C8gamma heterodimer and a noncovalently associated C8beta chain. C8 is the next component to bind the C5-7 complex forming C5b-8 that binds COMPLEMENT C9 and acts as a catalyst in the polymerization of C9.

The first complement component to act in the activation of CLASSICAL COMPLEMENT PATHWAY. It is a calcium-dependent trimolecular complex made up of three subcomponents: COMPLEMENT C1Q; COMPLEMENT C1R; and COMPLEMENT C1S at 1:2:2 ratios. When the intact C1 binds to at least two antibodies (involving C1q), C1r and C1s are sequentially activated, leading to subsequent steps in the cascade of COMPLEMENT ACTIVATION.

Molecular sites on or in some B-lymphocytes and macrophages that recognize and combine with COMPLEMENT C3B. The primary structure of these receptors reveal that they contain transmembrane and cytoplasmic domains, with their extracellular portion composed entirely of thirty short consensus repeats each having 60 to 70 amino acids.

An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).

The larger fragment generated from the cleavage of C5 by C5 CONVERTASE that yields COMPLEMENT C5A and C5b (beta chain + alpha' chain, the residual alpha chain, bound by disulfide bond). C5b remains bound to the membrane and initiates the spontaneous assembly of the late complement components to form C5b-8-poly-C9, the MEMBRANE ATTACK COMPLEX.

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

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

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

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

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

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.

Molecular sites on or in B-lymphocytes, follicular dendritic cells, lymphoid cells, and epithelial cells that recognize and combine with COMPLEMENT C3D. Human complement receptor 2 (CR2) serves as a receptor for both C3dg and the gp350/220 glycoprotein of HERPESVIRUS 4, HUMAN, and binds the monoclonal antibody OKB7, which blocks binding of both ligands to the receptor.

Serum peptides derived from certain cleaved COMPLEMENT PROTEINS during COMPLEMENT ACTIVATION. They induce smooth MUSCLE CONTRACTION; mast cell HISTAMINE RELEASE; PLATELET AGGREGATION; and act as mediators of the local inflammatory process. The order of anaphylatoxin activity from the strongest to the weakest is C5a, C3a, C4a, and C5a des-arginine.

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.

Multisubunit enzymes that reversibly synthesize ADENOSINE TRIPHOSPHATE. They are coupled to the transport of protons across a membrane.

The mitochondria of the myocardium.

Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.

The various filaments, granules, tubules or other inclusions within mitochondria.

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.

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.

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.

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.

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

The 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.

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

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

Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.

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

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

Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.

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

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.

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

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)

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

The rate dynamics in chemical or physical systems.

Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.

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

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

An adrenal microsomal cytochrome P450 enzyme that catalyzes the 21-hydroxylation of steroids in the presence of molecular oxygen and NADPH-FERRIHEMOPROTEIN REDUCTASE. This enzyme, encoded by CYP21 gene, converts progesterones to precursors of adrenal steroid hormones (CORTICOSTERONE; HYDROCORTISONE). Defects in CYP21 cause congenital adrenal hyperplasia (ADRENAL HYPERPLASIA, CONGENITAL).

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

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.

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

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

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

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

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

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

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

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

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

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

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.

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.

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 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.

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.

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

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.

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.

Established cell cultures that have the potential to propagate indefinitely.

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

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

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

The 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).

Proteins prepared by recombinant DNA technology.

An adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.

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.

An enzyme that catalyzes the HYDROLYSIS of the N-glycosidic bond between sugar phosphate backbone and URACIL residue during DNA synthesis.

Proteins found in plants (flowers, herbs, shrubs, trees, etc.). The concept does not include proteins found in vegetables for which VEGETABLE PROTEINS is available.

Enzymes which catalyze the endohydrolysis of 1,4-beta-D-xylosidic linkages in XYLANS.

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.

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.

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 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.

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

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.

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.

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.

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.

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).

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.

Proteins found in any species of bacterium.

Proteins that bind to and transfer CHOLESTEROL ESTERS between LIPOPROTEINS such as LOW-DENSITY LIPOPROTEINS and HIGH-DENSITY LIPOPROTEINS.

The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.

A 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)

The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.

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.

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.

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.

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.

A deoxyribonucleotide polymer that is the primary genetic material of all cells. Eukaryotic and prokaryotic organisms normally contain DNA in a double-stranded state, yet several important biological processes transiently involve single-stranded regions. DNA, which consists of a polysugar-phosphate backbone possessing projections of purines (adenine and guanine) and pyrimidines (thymine and cytosine), forms a double helix that is held together by hydrogen bonds between these purines and pyrimidines (adenine to thymine and guanine to cytosine).

A class of enzymes involved in the hydrolysis of the N-glycosidic bond of nitrogen-linked sugars.

Serine proteinase inhibitors which inhibit trypsin. They may be endogenous or exogenous compounds.

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.

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.

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.

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

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.

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.

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.

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

Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.

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.

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

A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.

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

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

Histochemical localization of immunoreactive substances using labeled antibodies as reagents.

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.

The process of cleaving a chemical compound by the addition of a molecule of water.

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.

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.

Compounds which inhibit or antagonize biosynthesis or actions of proteases (ENDOPEPTIDASES).

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

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

In vitro method for producing large amounts of specific DNA or RNA fragments of defined length and sequence from small amounts of short oligonucleotide flanking sequences (primers). The essential steps include thermal denaturation of the double-stranded target molecules, annealing of the primers to their complementary sequences, and extension of the annealed primers by enzymatic synthesis with DNA polymerase. The reaction is efficient, specific, and extremely sensitive. Uses for the reaction include disease diagnosis, detection of difficult-to-isolate pathogens, mutation analysis, genetic testing, DNA sequencing, and analyzing evolutionary relationships.

Proteins to which calcium ions are bound. They can act as transport proteins, regulator proteins, or activator proteins. They typically contain EF HAND MOTIFS.

Antibodies produced by a single clone of cells.

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.

Presence of warmth or heat or a temperature notably higher than an accustomed norm.

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.

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.

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.

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)

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

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

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.

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.

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

A family of enzymes that catalyze the conversion of ATP and a protein to ADP and a phosphoprotein.

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.

The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)

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 on non-ionic gels without regard to the mechanism of solute discrimination.

A proton ionophore that is commonly used as an uncoupling agent in biochemical studies.

Agents that inhibit PROTEIN 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.

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.

Enzymes that catalyze the hydrolysis of ester bonds within RNA. EC 3.1.-.

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.

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.)

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.

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

The 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 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.

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

A large 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.

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

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.

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

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.

The biosynthesis of RNA carried out on a template of DNA. The biosynthesis of DNA from an RNA template is called REVERSE TRANSCRIPTION.

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.

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)

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.

The chemical and physical integrity of a pharmaceutical product.

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

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

Proteins 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.

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.

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.

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.

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.

The relationship between the dose of an administered drug and the response of the organism to the drug.

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.

The process by which two molecules of the same chemical composition form a condensation product or polymer.

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).

A characteristic feature of enzyme activity in relation to the kind of substrate on which the enzyme or catalytic molecule reacts.

Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.

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)

Acute angioedema is usually triggered by an allergic reaction or exposure to certain medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs), blood pressure medications, or antibiotics. It can also be caused by infections, insect bites, and other environmental triggers.

Chronic angioedema, on the other hand, is a more persistent form of the condition that can last for weeks, months, or even years. It is often associated with conditions such as hereditary angioedema (HAE), which is caused by a genetic defect that affects the production of a protein called C1 esterase inhibitor.

The symptoms of angioedema can vary depending on the location and severity of the swelling, but they typically include:

* Swelling in the face, hands, feet, or other parts of the body
* Redness and warmth of the affected area
* Pain or discomfort
* Difficulty breathing or swallowing (in severe cases)

There is no cure for angioedema, but there are several treatments available to help manage the symptoms. These may include:

* Antihistamines or corticosteroids to reduce inflammation and relieve itching
* Ice packs or cool compresses to reduce swelling
* Compression stockings or bandages to prevent fluid buildup
* Pain relief medications, such as ibuprofen or acetaminophen, to manage discomfort

In severe cases of angioedema, hospitalization may be necessary to provide more intensive treatment and monitoring. In some cases, injectable medications such as epinephrine or corticosteroids may be administered to help reduce swelling and prevent complications.

Overall, angioedema is a serious condition that requires prompt medical attention to manage symptoms and prevent complications. If you suspect you or someone else may have angioedema, it is important to seek medical help right away.

There are two main types of hemolysis:

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

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

Some common causes of hemolysis include:

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

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

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

There are several subtypes of LES, including:

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

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

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

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

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

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

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

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

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

The main complications of GNM include:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Lupus Nephritis can cause a range of symptoms, including:

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

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

There are several key features of inflammation:

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

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

There are several types of inflammation, including:

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

There are several ways to reduce inflammation, including:

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

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

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

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

There are three main forms of ACH:

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

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

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

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

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

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

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

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

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

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

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

There are two main types of MD:

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

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

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

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

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

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

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

There are several types of disease susceptibility, including:

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

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

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

There are several symptoms of RA, including:

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

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

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

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

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

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

Examples of diseases with a known genetic predisposition:

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

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

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

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

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

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

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

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

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

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

... named C1-inhibitor. The inhibition of C1-inhibitor leads to over-activation of the complement pathway and one protein that ... Also, there has been some speculation as to an additional autoantibody against an inhibitor protein (in the complement pathway ... Consequently, levels of all complement proteins become low. The complement pathway is composed of several subset pathways: the ... C1q is an integral component within the complement pathway - a complicated cascade of protein interactions, culminating in an ...

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

Serum albumin Complement proteins C1-inhibitor C3-convertase Factor VIII Factor XIII Protein C Protein S Protein Z Protein Z- ... 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 ... Biology Enzymes Genes Proteins Index of protein-related articles List of enzymes NPW PEGylation Protein design Enzymes Protein ...

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

... the first component of the classical complement pathway C1 domain, an important secondary messenger protein domain C1-inhibitor ... Look up c1 in Wiktionary, the free dictionary. C1, C01, C.I or C-1 may refer to: C1, a note-octave in music C1 Television, a ... a precursor protein to Cytochrome C Proanthocyanidin C1, a type of polyphenolic compound Prostaglandin C1, a form of ... a human serine protease inhibitor C1 regulatory sequence for the insulin gene Apolipoprotein C1, a human lipoprotein ...

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

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. ... "Complement Deficiencies. What are complement deficiencies?". patient.info. Retrieved 31 December 2017. "Complement Deficiencies ...

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

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 ... All plasma proteins except Gamma-globulins are synthesised in the liver. Human serum albumin, osmolyte and carrier protein α- ... Vitamin D-binding protein, carries vitamin D FGF21, a protein hormone that induces mitochondrial oxidation of fatty acids, ...

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

C1 inhibitor (C1-INH) antigenic protein, C1 inhibitor (C1-INH) functional level if available. Analysis of complement 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 ... Other treatment modalities can stimulate the synthesis of C1 inhibitor, or reduce C1 inhibitor consumption. Purified C1 ...

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

... 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 ...

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

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- ...

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

... 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 ...

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

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, ...

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

MASP2 deficiency Complement receptor 3 deficiency Membrane cofactor protein (CD46) deficiency Membrane attack complex inhibitor ... C1-inhibitor deficiency (hereditary angioedema) Factor I deficiency (pyogenic infections) Factor H deficiency (haemolytic- ... Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to ... These proteins, generated by plasma cells, normally bind to pathogens, targeting them for destruction. Absent B cells with a ...

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

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 ...

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

... (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. ... C1q - another part of the C1 complex C1r - another part of the C1 complex MASP-2 - a protein similar to C1s, part of the lectin ... "Human C1 inhibitor: primary structure, cDNA cloning, and chromosomal localization". Biochemistry. 25 (15): 4292-301. doi: ...

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

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 ...

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

The classical pathway is inhibited by C1-inhibitor, which binds to C1 to prevent its activation. C3-convertase can be inhibited ... About 50 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 blood plasma and host cell membrane. ...

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

... complement c1 inactivator proteins MeSH D12.776.124.486.274.920.250.500 - complement c1 inhibitor protein MeSH D12.776.124.486. ... complement c1 MeSH D12.776.124.486.274.050.270 - complement c1q MeSH D12.776.124.486.274.050.280 - complement c1r MeSH D12.776. ... complement factor i MeSH D12.776.124.486.274.920.662 - complement c4b-binding protein MeSH D12.776.124.486.274.930 - complement ... complement c3b inactivator proteins MeSH D12.776.124.486.274.920.325.200 - complement factor h MeSH D12.776.124.486.274.920. ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776.124)

The role of C1-INH is to regulate and control the activities of the complement cascade, such that complement proteins remain in ... The C1 esterase inhibitor (C1-INH) enzyme plays a role in the classical pathway of the complement cascade, which is a component ... low C1-INH protein level, low C1q level, and decreased C1-INH protein function. Using the diagnostic approach mentioned here ... then downstream proteins in the complement cascade are activated, including complement component 2 (C2), complement component 3 ...

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

Six mutations of the gene SERPING1 (Serpin Peptidase Inhibitor, Clade G (C1 Inhibitor), Member 1) are associated with AMD. ... on chromosome 6 at 6p21.3 Polymorphisms in genes for complement system proteins: Variation in the genes for the complement ... Complement factor H (CFH) is an important inhibitor of this inflammatory cascade, and a disease-associated polymorphism in the ... While there is increasing academic and pharmaceutical interest in developing complement inhibitors to treat ophthalmic ...

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

Checkpoint inhibitors (CTLA-4, PD-1, and PD-L1) operate by this mechanism. Briefly, checkpoint inhibitors are proteins that ... Complement-dependent cytotoxicity occurs when antibodies bind to the cancer cell surface, the C1 complex binds to these ... The complement system includes blood proteins that can cause cell death after an antibody binds to the cell surface (the ... Checkpoint inhibitors bind these proteins and prevent them from functioning normally, which increases the activity of the ...

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

... 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 ...

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

APOE qualifies as a checkpoint inhibitor of the classical complement pathway by complex formation with activated C1q. ... Proteins similar in function have been found in choanoflagellates, suggesting that they are a very old class of proteins ... The gene, APOE, is mapped to chromosome 19 in a cluster with apolipoprotein C1 (APOC1) and the apolipoprotein C2 (APOC2). The ... Apolipoprotein E (APOE) is a protein involved in the metabolism of fats in the body of mammals. A subtype is implicated in ...

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

... 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 ...

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

In addition to the generation of complement proteins, C1 complex also induces the activation of B cells, monocytes, macrophages ... also called immune checkpoints inhibitors, obstruct the binding between cancer cells and immune checkpoints to antagonize ... followed by the cleavage of C3 protein into C3a and C3b protein. C3a protein serves as an inflammation mediator to recruit ... Briefly, the C1 protein attaches to the pathogen surface and the antibody-antigen complex that culminates in the generation of ...

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

... 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 ... The serpin barley protein Z is highly abundant in barley grain, and one of the major protein components in beer. The genome of ...

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

"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". ...

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

C6 C7 C8 C9 Complement pathway inhibitors C1-inhibitor - Classical, Lectin, Alternate Decay-accelerating factor (CD59) - ... 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 (PGRPs) PGLYRP1 PGLYRP2 PGLYRP3 PGLYRP4 Ficolins FCN1 FCN2 FCN3 Complement ...

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

When a single gene deficiency does cause lupus, it is usually attributed to the complement protein genes C1, C2, or C4. The ... Corticosteroids Antimalarial drugs BLyS-specific inhibitors Immunosuppressive agents/chemotherapy After being diagnosed some ... cutaneous lupus mucinosis complement deficiency syndromes drug-induced lupus erythematosus neonatal lupus erythematosus ...

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

Because of this motif-specific behavior, the enzyme also acts on other proteins that contain this same sequence. Such proteins ... Müller W, Hanauske-Abel H, Loos M (October 1978). "Biosynthesis of the first component of complement by human and guinea pig ... HIF prolyl-hydroxylase has been targeted by a variety of inhibitors that aim to treat stroke, kidney disease, ischemia, anemia ... peritoneal macrophages: evidence for an independent production of the C1 subunits". Journal of Immunology. 121 (4): 1578-84. ...

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

... wnt1 protein MeSH D12.776.624.664.700.978 - wnt2 protein MeSH D12.776.624.776.355.100 - cyclin-dependent kinase inhibitor p15 ... lupus coagulation inhibitor MeSH D12.776.377.715.548.114.323.300 - complement c3 nephritic factor MeSH D12.776.377.715.548.114. ... cytochromes c1 MeSH D12.776.422.220.286.300 - cytochromes c2 MeSH D12.776.422.220.286.600 - cytochromes c6 MeSH D12.776.422.220 ... groel protein MeSH D12.776.602.500.500.100 - fusion proteins, bcr-abl MeSH D12.776.602.500.500.320 - fusion proteins, gag-onc ...

https://en.wikipedia.org/wiki/List_of_MeSH_codes_(D12.776)

The Factor H-related protein 1 (FHR1) has been identified as a novel inhibitor of the complement pathway. FHR1 blocks C5 ... C2a produced by cleavage mediated by C1 complex, and C3b produced by cleavage mediated by the classical pathway C3 convertase ( ... The target of C5 convertase is complement protein C5. C5 is a two-chain (α, β) plasma glycoprotein (Mr = 196,000). C5 and C3 ... 2009). "Factor H-related protein 1 (FHR-1) inhibits complement C5 convertase activity and terminal complex formation". Blood. ...

https://en.wikipedia.org/wiki/C5-convertase

The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct ... The GPI inhibitors ERI4P and 6PG were found to block metastasis of BTS but not BTS glycolysis or fibroblast viability. In ... To complete the isomerization, Glu357 donates its proton to C1, the C2 hydroxyl group loses its proton and the open-chain ... arthritis without reactive oxygen species and complement". The American Journal of Pathology. 183 (4): 1144-1155. doi:10.1016/j ...

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

Acquired C1 esterase inhibitor deficiency Acute urticaria Adrenergic urticaria Anaphylaxis Aquagenic urticaria Cholinergic ... Complement deficiency DiGeorge syndrome (DiGeorge anomaly, thymic hypoplasia) Graft-versus-host disease Griscelli syndrome ... cutaneous neoplasms associated with systemic syndromes List of cutaneous conditions caused by problems with junctional proteins ... Childhood dermatomyositis Childhood discoid lupus erythematosus Childhood systemic lupus erythematosus Complement deficiency ...

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

PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... HRG Thrombophilia due to protein C deficiency, autosomal dominant; 176860; PROC Thrombophilia due to protein C deficiency, ... type C1; 257220; NPC1 Niemann-Pick disease, type C2; 607625; NPC2 Niemann-Pick disease type D; 257220; NPC1 Night blindness, ... Lutheran inhibitor; 111150; KLF1 Bloom syndrome; 210900; RECQL3 Blue cone monochromacy; 303700; OPN1MW Blue cone monochromacy; ...

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

Apfeld J, O'Connor G, McDonagh T, DiStefano PS, Curtis R (December 2004). "The AMP-activated protein kinase AAK-2 links energy ... Scientists show that and how the flavonoid Procyanidin C1 of the antioxidant grape seed extract increases the health- and ... glucosidase inhibitor or a Nrf2‐inducer". Aging Cell. 15 (5): 872-884. doi:10.1111/acel.12496. PMC 5013015. PMID 27312235. ... complementing similar results of a 2020 study. A scientific review concludes that accumulating data suggests dietary ...

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

Effector functions also require the use of complement proteins in serum or Fc-receptor on cell membranes. Ansuvimab has been ... August 2011). "Small molecule inhibitors reveal Niemann-Pick C1 is essential for Ebola virus infection". Nature. 477 (7364): ... would interact with virus's cell receptor protein, Niemann-Pick C1 (NPC1). This "competition" by ansuvimab prevents Ebola virus ... Ansuvimab is a neutralizing antibody, meaning it binds to a protein on the surface of Ebola virus that is required to infect ...

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

... is a protein that in humans is encoded by the DCN gene. Decorin is a proteoglycan that is on average 90 - 140 ... Decorin appears to influence fibrillogenesis, and also interacts with fibronectin, thrombospondin, the complement component C1q ... "The proteoglycan decorin binds C1q and inhibits the activity of the C1 complex". Journal of Immunology. 149 (11): 3695-701. ... "Localization of transforming growth factor beta and its natural inhibitor decorin in the human placenta and decidua throughout ...

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

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

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

Complement regulatory protein C1 inhibitor binds to selectins and interferes with endothelial-leukocyte adhesion. ... Complement regulatory protein C1 inhibitor binds to selectins and interferes with endothel ... C1 inhibitor (C1INH), a member of the serine proteinase inhibitor (serpin) family, is an inhibitor of proteases in the ... Coleções: Bases de dados internacionais Base de dados: MEDLINE Assunto principal: Complemento C1 / Endotélio Vascular / ...

https://pesquisa.bvsalud.org/portal/resource/pt/mdl-14568956

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

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

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

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

Complement C1 Inhibitor Protein / therapeutic use * Humans * Progestins / therapeutic use * Quality of Life ... There were 714 patients with C1 inhibitor (C1-INH) deficiency, of whom 423 (59.2%) were treated with LTP. Altered quality of ... Ongoing LTP included androgens (28.4%), progestins (25.8%), lanadelumab (25.3%), tranexamic acid (14.2%), intravenous C1-INHs ( ... 5.6%), and recombinant C1-INH (0.7%). Twenty-nine percent of the patents with LTP were considered to still have unmet needs. ...

https://pubmed.ncbi.nlm.nih.gov/35868842/

Successful pregnancy outcome after treatment with C1-inhibitor concentrate in a patient with hereditary angioedema and a ... Complement C1 Inhibitor Protein / therapeutic use* Actions. * Search in PubMed * Search in MeSH ... Efficacy and safety of an intravenous C1-inhibitor concentrate for long-term prophylaxis in hereditary angioedema. Craig T, ... Management of hereditary angioedema with C1-inhibitor concentrate during two successive pregnancies. Chan W, Berlin N, Sussman ...

https://pubmed.ncbi.nlm.nih.gov/22884590/

Plasma derived C1-INH is an established treatment for both on demand and prophylaxis of HAE. Conestat alf … ... C1-INH) deficiency is a debilitating and potentially lethal disease. Management includes on-demand treatment of angioedema and ... Complement C1 Inhibitor Protein / therapeutic use Actions. * Search in PubMed * Search in MeSH ... Recombinant human C1 esterase inhibitor for the treatment of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE ...

https://www.ncbi.nlm.nih.gov/pubmed/30021471

Complement C1 Inhibitor Protein D12.776.872.140.500. Complement Fixation Tests E5.478.594.760.150. Complement Hemolytic ... Protein Isoprenylation G6.535.797.500. (Replaced for 2008 by Protein Prenylation). Protein-Tyrosine-Phosphatase D8.811.277.352. ... HIV Envelope Protein gp160 D12.776.964.775.325.380 D12.776.964.775.325.164.374. D12.776.964.970.880.325.380 D12.776.964.775. ... Immediate-Early Proteins D12.776.260.900.610 D12.776.964.925.968. D12.776.964.950.610. Immune Adherence Reaction E5.478.525 ...

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

C1 Esterase Inhibitor C1-INH Protein C1-Inhibitor Protein Complement C1-Inhibitor Protein Plasma Protease C1 Inhibitor SERPING1 ... Proteins [D12.776] * Serpins [D12.776.872] * Complement C1 Inactivator Proteins [D12.776.872.140] * Complement C1 Inhibitor ... Complement C1 Inactivator Proteins [D12.644.861.140] * Complement C1 Inhibitor Protein [D12.644.861.140.500] ... Complement C1 Inactivator Proteins [D12.776.124.486.274.920.250] * Complement C1 Inhibitor Protein [D12.776.124.486.274.920. ...

https://meshb.nlm.nih.gov/record/ui?ui=D050718

4. Serum concentrations of C reactive protein, alpha1 antitrypsin, and complement (C3, C4, C1 esterase inhibitor) before and ... Circulating immune complexes, complement factors C3, C4, C1-inhibitor, alpha-1-antitrypsin and immunoglobulins in asthmatic ... C3 and C4 complement components and acute phase proteins in late pregnancy and parturition.. Kovar IZ; Riches PG. J Clin Pathol ... 9. Host serum protein levels in cysts of human hydatidosis.. Vidor E; Piens MA; Garin JP. Trans R Soc Trop Med Hyg; 1987; 81(4 ...

https://pubmedhh.nlm.nih.gov/biomarkers/search.php?id=12911163&mod=related&page=1&outid=&proj=

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

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

... "c1"[All Fields] AND "inhibitor"[All Fields] AND "protein"[All Fields]) OR "complement c1 inhibitor protein"[All Fields] OR ("c1 ... "complement c1 inhibitor protein"[MeSH Terms] OR ("complement"[All Fields] AND " ... "[All Fields] AND "esterase"[All Fields] AND "inhibitor"[All Fields]) OR "c1 esterase inhibitor"[All Fields]). Search. ...

https://ncbi.nlm.nih.gov/books/NBK501922/?term=HUMAN+C1-ESTERASE+INHIBITOR

C1-Inhibitor deficiency and angioedema. In: K. Whaley, ed. Complement in Health and Disease. MTP Press Limited; 1987;p 53. ... What is the difference between the C1 Inhibitor, Protein test and the C1 Inhibitor, Functional test? The C1 Inhibitor, Protein ... measures the concentration of the C1 esterase inhibitor protein using a fixed rate time nephelometry method. The C1 Inhibitor, ... Concentrations of the C1 inhibitor protein are normal (11-26 ng/mL), but the protein is dysfunctional (functional activity ,40 ...

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

https://meshb-prev.nlm.nih.gov/record/ui?ui=D050718

C1 Esterase Inhibitor C1 INH Protein C1 Inhibitor Protein C1-INH Protein C1-Inhibitor Protein Complement C1-Inhibitor Protein ... C1 Esterase Inhibitor. C1 INH Protein. C1 Inhibitor Protein. C1-INH Protein. C1-Inhibitor Protein. Complement C1-Inhibitor ... for C1 ESTERASE INHIBITOR see COMPLEMENT C1 INACTIVATOR PROTEINS. History Note:. 2006; for C1 ESTERASE INHIBITOR use COMPLEMENT ... Complement C1 Inhibitor Protein - Preferred Concept UI. M0476632. Scope note. An endogenous 105-kDa plasma glycoprotein ...

https://decs.bvsalud.org/en/ths/resource/?id=50836

https://decs2008.bvsalud.org/P/mnchg2008.htm

The C1 esterase inhibitor protein is required to control the complement and contact systems, collections of proteins in the ... is a copy of the C1 esterase inhibitor protein and works in the same way as the natural human protein. When it is given during ... Ruconest is used in patients with hereditary angioedema that is linked to naturally low levels of a protein called C1 esterase ... involving a total of 70 adults and adolescents with hereditary angioedema caused by low levels of C1 esterase inhibitor protein ...

https://www.ema.europa.eu/en/medicines/human/EPAR/ruconest

Complement C1 N0000170005 Complement C1 Inactivator Proteins N0000169305 Complement C1 Inhibitor Protein N0000169277 Complement ... Wnt Proteins N0000170952 Wnt1 Protein N0000170953 Wnt2 Protein N0000169101 WT1 Proteins N0000170496 X-Linked Inhibitor of ... C3d N0000169282 Complement C4 N0000169290 Complement C4a N0000169283 Complement C4b N0000169308 Complement C4b-Binding Protein ... Protein C N0000170002 Protein C Inhibitor N0000168185 Protein D-Aspartate-L-Isoaspartate Methyltransferase N0000167798 Protein ...

https://evs.nci.nih.gov/ftp1/FDA/ndfrt/Archive/StructuralClass%202007-10-09.txt

HN - 2010 FX - Complement C1 Inhibitor Protein MH - Hereditary Autoinflammatory Diseases UI - D056660 MN - C16.320.382 MN - ... Type I hereditary angioedema is associated with reduced serum levels of complement C1 inhibitor protein. Type II hereditary ... Forms of hereditary angioedema that occur due to mutations in the gene for COMPLEMENT C1 INHIBITOR PROTEIN. ... angioedema is associated with the production of a non-functional complement C1 inhibitor protein. ...

https://nlmpubs.nlm.nih.gov/projects/mesh/.newterms/mshnew2010.txt

Alternative PathwayComplement C1 Inhibitor ProteinImmunoglobulin GComplement C3 Convertase, Alternative PathwayComplement C5 ... Complement C3Complement C4Complement C4aComplement C3aComplement C1qComplement C5aComplement C4bComplement C5Complement C3b ... Complement C3Complement C4Complement C4aComplement C3aComplement C1qComplement C5aComplement ActivationComplement C4bComplement ... Complement System ProteinsComplement C6Complement C3cComplement C3dComplement C2Complement C9Receptors, ComplementComplement ...

https://lookformedical.com/en/search/complement-c7

Complement C1 Inactivator Proteins D12.644.822.750.140 D12.776.645.750.140 Complement C1 Inhibitor Protein D12.644.822.750. ... Protein Array Analysis E5.196.630.570.700 E5.393.525.680 Protein C Inhibitor D12.644.822.750.695.700 D12.776.645.750.695.700 ... Son of Sevenless Protein, Drosophila D12.776.402.300.700.700.600 Son of Sevenless Proteins D12.776.402.300.700.700 SOS1 Protein ... GTPase-Activating Proteins D12.776.402.150 Guanine Nucleotide Dissociation Inhibitors D12.776.402.225 Guanine Nucleotide ...

https://decs2018.bvsalud.org/P/mnchg2016.txt

https://decs2016.bvsalud.org/P/mnchg2016.txt

https://decs2017.bvsalud.org/P/mnchg2016.txt

C1-inhibitor, complement inhibitor C4b-binding protein, decay-activating factor, and factor H. When these regulatory mechanisms ... Yasojima K, McGeer EG, McGeer PL, Complement regulators C1 inhibitor and CD59 do not significantly inhibit complement ... C1q (the first protein in the classic complement pathway), early complement activation proteins (C4 and C3 activation fragments ... native complement proteins are enzymatically cleaved, generating complement activation proteins that function as opsinins, ...

https://touchneurology.com/alzheimers-disease-dementia/journal-articles/significance-of-complement-activation-in-alzheimers-disease/

Cholesterol Ester Transfer Proteins. *Clusterin. *Colony-Stimulating Factors. *Complement C1 Inhibitor Protein ...

https://uams-triprofiles.uams.edu/profiles/display/109677

HAEGARDA contains C1 esterase inhibitor (C1-INH), a protein that helps control C1. ... C1-INH is the only known inhibitor for the C1r and C1s subcomponents of complement component 1 (C1), coagulation factor XIIa, ... HUMAN C1-ESTERASE INHIBITOR (UNII: 6KIC4BB60G) (HUMAN C1-ESTERASE INHIBITOR - UNII:6KIC4BB60G) HUMAN C1-ESTERASE INHIBITOR. ... HUMAN C1-ESTERASE INHIBITOR (UNII: 6KIC4BB60G) (HUMAN C1-ESTERASE INHIBITOR - UNII:6KIC4BB60G) HUMAN C1-ESTERASE INHIBITOR. ...

https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=e270ad38-adc4-4ed5-88e3-943b93d20a57&audience=consumer

Antibodies against several complement components and their regulatory proteins, like the C1-inhibitor, have been detected in ... Therefore, the three different activation pathways of the complement system and the soluble terminal complement complex (sTCC) ... The correlation between SLEDAI-score, complement activation capacity, and sTCC concentration suggests consumption of complement ... while activation of complement proteins is associated with disease activity [1, 8-10]. Current laboratory diagnostic includes ...

https://biogenericpublishers.com/jbgsr-ms-id-00216-text/

C1 esterase inhibitors), known as complements. They help to regulate various body functions (e.g., flow of body fluids in and ... It occurs as the result of the production of abnormal complement proteins and accounts for about 15-20% of this disorder. ... Most of people with HAE acquire a C1 esterase inhibitor (C1-INH) mutation from one of their parents. A parent with HAE usually ... The underlying cause of HAE is attributed to autosomal dominant inheritance of mutations in the C1 inhibitor (C1-INH gene or ...

https://allaboutheaven.org/science/hereditary-angioedema/121

Cholesterol Ester Transfer Proteins. *Clusterin. *Colony-Stimulating Factors. *Complement C1 Inhibitor Protein ... "HSP47 Heat-Shock Proteins" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical ... This graph shows the total number of publications written about "HSP47 Heat-Shock Proteins" by people in this website by year, ... Below are the most recent publications written about "HSP47 Heat-Shock Proteins" by people in Profiles. ...

https://profiles.ouhsc.edu/display/20014

The aim of this study is to assess the long-term safety of C1-esterase inhibitor (C1-INH) in preventing hereditary angioedema (HAE) attacks when it is administered under the skin of subjects with HAE. (clinicaltrials.gov)
4. Serum concentrations of C reactive protein, alpha1 antitrypsin, and complement (C3, C4, C1 esterase inhibitor) before and during the Vuelta a Espańa. (nih.gov)
The C1 esterase inhibitor protein is required to control the 'complement' and 'contact' systems, collections of proteins in the blood that fight against infection and cause inflammation. (europa.eu)
The active substance in Ruconest, conestat alfa, is a copy of the C1 esterase inhibitor protein and works in the same way as the natural human protein. (europa.eu)
Ruconest was studied in two main studies involving a total of 70 adults and adolescents with hereditary angioedema caused by low levels of C1 esterase inhibitor protein. (europa.eu)
HAE type I - The most common form of the disorder is HAE type I, which is the result of abnormally low levels of certain complex proteins in the blood (C1 esterase inhibitors), known as complements. (allaboutheaven.org)
Most of people with HAE acquire a C1 esterase inhibitor (C1-INH) mutation from one of their parents. (allaboutheaven.org)
HAEGARDA is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) (C1-INH) indicated for routine prophylaxis to prevent Hereditary Angioedema (HAE) attacks in patients 6 years of age and older. (nih.gov)
C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood. (medlineplus.gov)
Your health care provider will also measure the functional activity level of your C1 esterase inhibitor. (medlineplus.gov)
Types I and II are clinically identical, involving deficiency or malfunction of the protein C1 inhibitor (C1-INH) - also called C1 esterase inhibitor. (patient.info)
Angioedema is associated with the following disorders: 1) acquired C1-esterase inhibitor deficiency and 2) hereditory C1-esterase inhibitor deficiency. (mhmedical.com)
HAE type I, which accounts for approximately 85% of patients is the result of decreased C1-esterase inhibitor production. (mhmedical.com)
The remainder 15% of patients suffer from HAE type II, which is characterized by normal or even elevated levels of a functionally impaired C1-esterase inhibitor. (mhmedical.com)
The hereditary form is autosomal dominant transmitted and caused by a mutation in the C1 inhibitor gene (C1-esterase inhibitor) located on chromosome 11p11.2-q13.1. (mhmedical.com)
Activation leads to cleavage producing the C1 esterase, which may now act on C4 and C2. (mhmedical.com)
C1-esterase inhibitor is the control protein that inhibits the spontaneous activation. (mhmedical.com)
C1-INH is also called C1 esterase inhibitor, due C1s is often cleaved by synthetic esters in spectrophotometry. (eaglebio.com)

There were 714 patients with C1 inhibitor (C1-INH) deficiency, of whom 423 (59.2%) were treated with LTP. (nih.gov)
Hereditary angioedema (HAE) due to C1 inhibitor (C1-INH) deficiency is a debilitating and potentially lethal disease. (nih.gov)
Areas covered: This review will critically appraise available information about rhC1-INH (conestat alfa) prophylactic treatment in adult and adolescent patients with congenital C1-INH deficiency. (nih.gov)
The underlying cause of HAE is attributed to autosomal dominant inheritance of mutations in the C1 inhibitor (C1-INH gene or SERPING1 gene), which is mapped to chromosome 11 (11q12-q13.1).To date there are over 300 known genetic mutations that result in a deficiency of functional C1 Inhibitor. (allaboutheaven.org)
Rarely, people may inherit deficiency of some complement proteins. (medlineplus.gov)
Diagnosis, course, and management of angioedema in patients with acquired C1-inhibitor deficiency. (medlineplus.gov)
Cases of complement deficiency have helped defined the role of complement in host defense. (medscape.com)
A North African study of molecular basis of complement factor I deficiency in atypical hemolytic and uremic syndrome patients suggested that the Ile357Met mutation may be a founding effect. (medscape.com)
C1‐INH deficiency can be acquired, or there are other causes. (patient.info)
A disease commonly caused by heterozygous deficiency of C1-INH and leading to low levels of functional C1-INH and recurrent episodes of dermal and submucosal swelling. (eaglebio.com)

Management of hereditary angioedema with C1-inhibitor concentrate during two successive pregnancies. (nih.gov)
19. Protein sgp 120 as a marker of an acquired angioedema. (nih.gov)
Los individuos con déficit de C1-INH sufren ANGIOEDEMA HEREDITARIO. (bvsalud.org)
Patients with low levels of this protein have excessive activity of these two systems, which leads to the symptoms of angioedema. (europa.eu)
The low level of C1 inhibitor in the plasma leads to increased activation of pathways that release bradykinin, the chemical responsible for the angioedema due to increased vascular permeability, and the pain seen in individuals with HAE. (allaboutheaven.org)
In a review of patients who do not have a history of HAE in their family, but who have relatively low levels of mutated C1-INH with persistent angioedema, 25% of new patients who had HAE had C1-INH changes that do not show signs of being inherited but rather new. (allaboutheaven.org)
Both forms of angioedema are caused by low levels of C1-INH. (medlineplus.gov)
Low levels of C1-INH may cause certain types of angioedema. (medlineplus.gov)
There are two types of angioedema that result from decreased levels of C1-INH. (medlineplus.gov)
C1-INH administration is the common treatment for hereditary angioedema (HAE). (eaglebio.com)
Hereditary angioedema (HAE) is an autosomal dominant disorder that involves a defect in the C1-INH protein. (symptoma.com)
Controlling prekallikrein activation by C1 inhibitor (C1Inh) represents the most essential mechanism for angioedema patient protection. (biomedfrontiers.org)
The measurement of C1 Inhibitor (C1Inh) is the core laboratory testing for angioedema diagnosis. (biomedfrontiers.org)

It is caused by a mutation in the C1 inhibitor - C1-INH gene or SERPING1 gene. (allaboutheaven.org)
Types I and II are caused by one of hundreds of possible different mutations in the SERPING1 gene, which codes for C1‐INH. (patient.info)

C1 inhibitor (C1INH), a member of the serine proteinase inhibitor ( serpin ) family , is an inhibitor of proteases in the complement system, the contact system of kinin generation, and the intrinsic coagulation pathway. (bvsalud.org)
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). (lookformedical.com)
C1 inhibitor function using contact-phase proteases as target: evaluation of an innovative assay. (biomedfrontiers.org)
Table I. Control of contact phase, fibrinolysis, and complement proteases by C1 Inhibitor and α2Macroglobulin. (biomedfrontiers.org)

There are 21666 SERPIN domains in 21322 proteins in SMART's nrdb database. (embl.de)
Taxonomic distribution of proteins containing SERPIN domain. (embl.de)
The complete taxonomic breakdown of all proteins with SERPIN domain is also avaliable . (embl.de)
Click on the protein counts, or double click on taxonomic names to display all proteins containing SERPIN domain in the selected taxonomic class. (embl.de)
Because the deduced amino acid sequence qualified the protein as a novel member of the serpin family of serine protease inhibitors, we called it neuroserpin. (embl.de)

5. Evidence for the involvement of arginine 462 and the flanking sequence of human C4 beta-chain in mediating C5 binding to the C4b subcomponent of the classical complement pathway C5 convertase. (nih.gov)
7. A single arginine to tryptophan interchange at beta-chain residue 458 of human complement component C4 accounts for the defect in classical pathway C5 convertase activity of allotype C4A6. (nih.gov)
11. Distal recognition site for classical pathway convertase located in the C345C/netrin module of complement component C5. (nih.gov)
A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. (lookformedical.com)
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. (lookformedical.com)
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. (lookformedical.com)
C1q (the first protein in the classic complement pathway), early complement activation proteins (C4 and C3 activation fragments), and the MAC have been demonstrated by immunocytochemical staining in the Alzheimer's disease (AD) brain on senile plaques, NFTs, neuropil threads, and dystrophic neurites 9-12 (see Figure 2 ). (touchneurology.com)
Aggregated Aβ efficiently binds C1q, activating the classic complement pathway, 2 and this process further enhances Aβ aggregation and fibril formation. (touchneurology.com)
Complement activation in AD was initially reported to be limited to the classic pathway, 9 but alternative pathway activation was later reported as well. (touchneurology.com)
Complement deficiencies, especially in the early classical pathway, predispose to the development of SLE, while activation of complement proteins is associated with disease activity [1, 8-10]. (biogenericpublishers.com)
The complement cascade consists of 3 separate pathways that converge in a final common pathway. (medscape.com)
The classical pathway is triggered by interaction of the Fc portion of an antibody (immunoglobulin [Ig] M, IgG1, IgG2, IgG3) or C-reactive protein with C1q. (medscape.com)
Lectins activate the lectin pathway in a manner similar to the antibody interaction with complement in the classical pathway. (medscape.com)
C1, the first component of the classical pathway of the complement system is a complex macromolecule. (mhmedical.com)
Factor D is unique to the Alternative Complement Pathway. (quidel.com)

1. Serum alpha1-antitrypsin but not complement C3 and C4 predicts chronic inflammation in hemodialysis patients. (nih.gov)
7. [Circadian rhythms of the so-called inflammation proteins in healthy subjects]. (nih.gov)
Complement activation leads to inflammation, and eventually, organ damages occur. (biogenericpublishers.com)
Anti-dsDNA antibodies and histones stimulate the circulation of immune complexes leading to inflammation, complement activation, and tissue damage [3]. (biogenericpublishers.com)
at the same time, the inflammation promoted by complement activation can result in cellular damage when not kept in check. (medscape.com)
C1-INH plays an important role in suppression of inflammation and vascular permeability. (eaglebio.com)

Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES. (lookformedical.com)

Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors. (umbc.edu)

There are three pathways of complement activation. (eaglebio.com)

Complement regulatory protein C1 inhibitor binds to selectins and interferes with endothelial-leukocyte adhesion. (bvsalud.org)
The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX. (lookformedical.com)

16. Cytokines associated with amyloid plaques in Alzheimer's disease brain stimulate human glial and neuronal cell cultures to secrete early complement proteins, but not C1-inhibitor. (nih.gov)

Serpins (SERine Proteinase INhibitors) belong to MEROPS inhibitor family I4, clan ID. (embl.de)
SERine Proteinase INhibitors (serpins), prokaryotic subgroup. (umbc.edu)

16. C3 and C4 complement components and acute phase proteins in late pregnancy and parturition. (nih.gov)

The smaller fragment generated from the cleavage of complement C3 by C3 CONVERTASE. (lookformedical.com)
C4a is a protein fragment from the cleavage of complement protein C4, along with C4b. (quidel.com)

C1-INH is part of the complement system, coagulation system and fibrinolytic system. (patient.info)

7 Complement activation is normally closely regulated through the actions of endogenous complement inhibitory proteins, 8 including CD59, clusterin, vitronectin, C1-inhibitor, complement inhibitor C4b-binding protein, decay-activating factor, and factor H. When these regulatory mechanisms are insufficient, then tissue damage can result. (touchneurology.com)

5. [Effect of a high saturated fatty acids load on serum concentrations of C-reactive protein, alpha1-antitrypsin, fibrinogen and alpha1-acid glycoprotein in obese women]. (nih.gov)
9. Host serum protein levels in cysts of human hydatidosis. (nih.gov)
14. The relationship between serum levels of lipoprotein(a) and proteins associated with the acute phase response. (nih.gov)
14. Effect of plicatic acid on human serum complement includes interference with C1 inhibitor function. (nih.gov)
Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. (lookformedical.com)
A 105-kDa serum glycoprotein with significant homology to the other late complement components, C7-C9. (lookformedical.com)
The complement system is composed of more than 30 plasma and membrane-associated proteins, accounting for approximately 10% of the globulins in vertebrate serum, which function as an inflammatory cascade. (touchneurology.com)
The Eagle Biosciences Human C1-INH ELISA Assay Kit is to be used for the in vitro quantitative determination of C1-INH in plasma and serum samples. (eaglebio.com)

13. Functional role of the linker between the complement control protein modules of complement protease C1s. (nih.gov)
20. Functional analysis of activated C1s, a subcomponent of the first component of human complement, by monoclonal antibodies. (nih.gov)
Analysis of the primary structural features further characterized neuroserpin as a heparin-independent, functional inhibitor of a trypsin-like serine protease. (embl.de)

Plasma derived C1-INH is an established treatment for both on demand and prophylaxis of HAE. (nih.gov)

Physicians' concerns varied among therapies: poor tolerability for androgens and progestins, a lack of efficacy for tranexamic acid and progestins, dosage form, and high costs for C1-INHs and lanadelumab. (nih.gov)

It is the most heavily glycosylated plasma protein , containing 13 definitively identified glycosylation sites as well as an additional 7 potential glycosylation sites. (bvsalud.org)
C1 inhibitor (C1-INH) is a heavily glycosylated single chain molecule of 500 AA. (eaglebio.com)

Some patients with type III HAE have a mutation in the F12 gene which produces a protein involved in blood clotting. (allaboutheaven.org)
Genes that encode the proteins of complement components or their isotypes are distributed throughout different chromosomes, with 19 genes comprising 3 significant complement gene clusters in the human genome. (medscape.com)

Therefore, the three different activation pathways of the complement system and the soluble terminal complement complex (sTCC) were analyzed. (biogenericpublishers.com)
SC5b-9 is the soluble version of the terminal complement complex (TCC). (quidel.com)

15 Whether elevated complement activation in AD may result, in part, from impaired local defense mechanisms is not clear, due to conflicting reports about the status of complement inhibitory proteins in the AD brain. (touchneurology.com)

Patient identification requires the determination of C1Inh function, this function is currently measured using C1 protease as target. (biomedfrontiers.org)
We developed a laboratory assay for C1Inh function using contact-phase proteins, the target involved in the pathological process, without interference of α2M or plasma protease. (biomedfrontiers.org)

20. Circulating immune complexes, complement factors C3, C4, C1-inhibitor, alpha-1-antitrypsin and immunoglobulins in asthmatic patients. (nih.gov)
Complement activation is a major inflammatory process whose primary functions are to assist in removing micro-organisms and cellular debris and processing of immune complexes. (touchneurology.com)
Complement can be activated by many factors, including immune complexes, polysaccharides (including lipopolysaccharide, the major component of the outer membrane of Gram-negative bacteria), and neuropathological structures such as senile plaques, neurofibrillary tangles (NFTs), and Lewy bodies. (touchneurology.com)
The complement proteins work with your immune system to protect the body from infections. (medlineplus.gov)
The complement system is part of the innate immune system. (medscape.com)
In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). (medscape.com)
New studies point to the complex interplay between the complement cascade and adaptive immune response, and complement is also being studied in association with ischemic injury as a target of therapy. (medscape.com)
The complement system plays important roles in both innate and adaptive immune response and can produce an inflammatory and protective reaction to challenges from pathogens before an adaptive response can occur. (eaglebio.com)

8. Molecular basis of complement resistance of human melanoma cells expressing the C3-cleaving membrane protease p65. (nih.gov)

As a part of the innate immunity, the complement system and its activation pathways (classical, lectin, and alternative pathways) play a complex role in SLE pathogenesis. (biogenericpublishers.com)
Although the complement system is part of the body's innate, relatively nonspecific defense against pathogens, its role is hardly primitive or easily understood. (medscape.com)
C1-INH spares the AP, leaving part of the innate antibacterial defense intact. (eaglebio.com)

The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells. (medlineplus.gov)
2007). The existing observations of AtECA:sGFP localization claim that these proteins could be involved with plasma membrane-associated procedures. (baxkyardgardener.com)
AtECA:sGFP protein localize primarily towards the plasma membrane in protoplasts. (baxkyardgardener.com)
AtECAs indicated as sGFP fusion protein localize towards the plasma membrane (PM), endosomes, and cell dish in transgenic vegetation. (baxkyardgardener.com)
We prevented any interference from a possible high plasma kininogenase activity by preincubating the samples with protease inhibitor. (biomedfrontiers.org)

The complement system plays an important part in defense against pyogenic organisms. (medscape.com)

Conestat alfa is a recombinant form of human C1-INH (rhC1-INH) produced in transgenic rabbits. (nih.gov)
Conestat alfa is produced by 'recombinant DNA technology': it is extracted from the milk of rabbits that have been given genes that make them able to produce the human protein in their milk. (europa.eu)

C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. (lookformedical.com)
The minor fragment formed when C5 convertase cleaves C5 into C5a and COMPLEMENT C5B. (lookformedical.com)
Each generates a C3 convertase, a serine protease that cleaves the central complement protein C3, and generates the major cleavage fragment C3b. (eaglebio.com)

However, thrombosis has occurred in treatment attempts with high doses of C1-INH intravenous (I.V.) for prevention or therapy of capillary leak syndrome before, during or after cardiac surgery (unapproved indication and dose). (nih.gov)

1. Complement component C5: engineering of a mutant that is specifically cleaved by the C4-specific C1s protease. (nih.gov)
4. Mutants of complement component C3 cleaved by the C4-specific C1-s protease. (nih.gov)
6. A 100-kDa protein in the C4-activating component of Ra-reactive factor is a new serine protease having module organization similar to C1r and C1s. (nih.gov)
9. The structure and function of the first component of complement: genetic engineering approach (a review). (nih.gov)
10. Identification of a catalytic exosite for complement component C4 on the serine protease domain of C1s. (nih.gov)
12. Structural homologies of component C5 of human complement with components C3 and C4 by neutron scattering. (nih.gov)
15. Murine complement component C4 and sex-limited protein: identification of amino acid residues essential for C4 function. (nih.gov)
18. Third component of trout complement. (nih.gov)
C6 is the next complement component to bind to the membrane-bound COMPLEMENT C5B in the assembly of MEMBRANE ATTACK COMPLEX. (lookformedical.com)

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

17. Complement C2 receptor inhibitor trispanning and the beta-chain of C4 share a binding site for complement C2. (nih.gov)
The important components of this system are various cell membrane-associated proteins such as complement receptor 1 (CR1), complement receptor 2 (CR2), and decay accelerating factor (DAF). (medscape.com)

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

When activation of the system occurs, native complement proteins are enzymatically cleaved, generating complement 'activation proteins' that function as opsinins, anaphylatoxins, and chemokines (see Table 1 ). (touchneurology.com)

C1-INH binding of C1 to the catalytic site of both C1r and C1s releases the latter two from the complex. (eaglebio.com)

This study aimed to investigate the involvement of the complement system in the pathophysiology of childhood-onset SLE. (biogenericpublishers.com)

This domain occurred 32 times on human genes ( 63 proteins). (umbc.edu)

8. Antiphospholipid Antibodies are Associated with Low Levels of Complement C3 and C4 in Patients with Systemic Lupus Erythematosus. (nih.gov)
sTCC concentration as well as the extent of activation capacity of the three complement pathways could serve as reliable parameters for monitoring the individual course of disease in paediatric SLE patients. (biogenericpublishers.com)
Do not use in patients with a history of life-threatening immediate hypersensitivity reactions, including anaphylaxis to C1-INH preparations or its excipients. (nih.gov)

Additionally, we studied the correlation between the systemic lupus erythematosus disease activity index (SLEDAI) and the complement-specific values measured at the time of blood withdrawal. (biogenericpublishers.com)
Complement factors may also be important in testing for autoimmune diseases, such as systemic lupus erythematosus. (medlineplus.gov)

The Human C1-INH ELISA Assay Kit is for research use only and not for diagnostic or therapeutic procedures. (eaglebio.com)
A rabbit antiserum raised against human complement C3a protein. (quidel.com)
A goat antiserum raised against human Factor D protein. (quidel.com)

2,3,12 Soluble, non-fibrillar Aβ may also be capable of activating complement, albeit to less of an extent than fibrillar Aβ. (touchneurology.com)

Increased mRNA levels for native complement proteins are also present. (touchneurology.com)
Current laboratory diagnostic includes complement protein levels (e.g. (biogenericpublishers.com)
Type I has low levels of C1-INH (the majority of cases). (patient.info)

Because complement activation exerts both protective and deleterious effects, it has been referred to as a 'double-edged sword. (touchneurology.com)
Complement activation is thought to be triggered in the AD brain primarily by the interaction of complement proteins with aggregated forms of amyloidbeta (Aβ) and tau protein, the major components in plaques and NFTs, respectively. (touchneurology.com)
14 Complement activation and plaque formation are mutually promoting mechanisms. (touchneurology.com)
18 The significance of complement activation in the development and progression of AD is unclear. (touchneurology.com)
Several of the activation proteins generated in this process have been demonstrated to exert neuroprotective effects in vitro , including protecting against excitotoxicity, 19 Aβ-induced neurotoxicity, 20 and apoptosis, 7 as well as facilitating the clearance of Aβ by microglia. (touchneurology.com)
As a result the activation of the complement system is blocked. (eaglebio.com)
The lack or non-functionality of said inhibitors leads to activation of an inflammatory cascade, which result in cutaneous and mucosal edema . (symptoma.com)

It occurs as the result of the production of abnormal complement proteins and accounts for about 15-20% of this disorder. (allaboutheaven.org)

Erythrocyte sedimentation rate, c-reactive-protein) [10]. (biogenericpublishers.com)

Besides, C1-INH can directly bind and neutralize LPS, inhibiting sepsis and endotoxin shock. (eaglebio.com)

CrmA rapidly inhibits ICE with an association rate constant (kon) of 1.7 x 10(7) M-1 s-1, forming a tight complex with an equilibrium constant for inhibition (Ki) of less than 4 x 10(-12) M. These data indicate that CrmA is a potent inhibitor of ICE, consistent with the dramatic effects of CrmA on modifying host responses to virus infection. (embl.de)

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

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

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)

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

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)

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

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

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

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Complement C3

Complement C4

Complement C4a

Complement C3a

Complement C1q

Complement C5a

Complement Activation

Complement C4b

Complement C5

Complement C3b

Complement System Proteins

Complement C6

Phosphatidylethanolamine Binding Protein

Complement C3c

Complement C3d

Complement C2

Complement C9

Receptors, Complement

Complement C1s

Complement Membrane Attack Complex

Complement C1r

Complement Inactivator Proteins

Complement C7

Complement C3-C5 Convertases

Complement Factor B

Complement Pathway, Alternative

Complement Pathway, Classical

Complement C1 Inactivator Proteins

Complement C8

Complement C1

Receptors, Complement 3b

Complement Factor H

Complement C5b

Complement C2a

Receptor, Anaphylatoxin C5a

Complement Activating Enzymes

Complement Inactivating Agents

Complement Hemolytic Activity Assay

Molecular Sequence Data

Receptors, Complement 3d

Anaphylatoxins

Amino Acid Sequence

Proton-Translocating ATPases

Mitochondria, Heart

Angioedema

Submitochondrial Particles

Complement Fixation Tests

Proteins

Aurovertins

Complement Factor D

Complement C1 Inhibitor Protein

Protein Binding

Complement Factor I

Complement C4b-Binding Protein

Complement C3b Inactivator Proteins

Antigens, CD55

Complement C3-C5 Convertases, Classical Pathway

Cattle

Complement C2b

Androgen-Binding Protein

Carrier Proteins

Antigens, CD59

Base Sequence

Kinetics

Enzyme Inhibitors

Cobra Venoms

Electrophoresis, Polyacrylamide Gel

Antigen-Antibody Complex

Steroid 21-Hydroxylase

Complement C3-C5 Convertases, Alternative Pathway

Alpha-Globulins

Binding Sites

Peptide Fragments

Immunoglobulin G

Hemolysis

Complement C3 Convertase, Alternative Pathway

Complement C5 Convertase, Classical Pathway

Sequence Homology, Amino Acid

Complement C3 Convertase, Classical Pathway

Antigens, CD46