Complement Factor I: A plasma serine proteinase that cleaves the alpha-chains of C3b and C4b in the presence of the cofactors COMPLEMENT FACTOR H and C4-binding protein, respectively. It is a 66-kDa glycoprotein that converts C3b to inactivated C3b (iC3b) followed by the release of two fragments, C3c (150-kDa) and C3dg (41-kDa). It was formerly called KAF, C3bINF, or enzyme 3b inactivator.Complement C3b: The larger fragment generated from the cleavage of COMPLEMENT C3 by C3 CONVERTASE. It is a constituent of the ALTERNATIVE PATHWAY C3 CONVERTASE (C3bBb), and COMPLEMENT C5 CONVERTASES in both the classical (C4b2a3b) and the alternative (C3bBb3b) pathway. C3b participates in IMMUNE ADHERENCE REACTION and enhances PHAGOCYTOSIS. It can be inactivated (iC3b) or cleaved by various proteases to yield fragments such as COMPLEMENT C3C; COMPLEMENT C3D; C3e; C3f; and C3g.Complement Factor H: An important soluble regulator of the alternative pathway of complement activation (COMPLEMENT ACTIVATION PATHWAY, ALTERNATIVE). It is a 139-kDa glycoprotein expressed by the liver and secreted into the blood. It binds to COMPLEMENT C3B and makes iC3b (inactivated complement 3b) susceptible to cleavage by COMPLEMENT FACTOR I. Complement factor H also inhibits the association of C3b with COMPLEMENT FACTOR B to form the C3bB proenzyme, and promotes the dissociation of Bb from the C3bBb complex (COMPLEMENT C3 CONVERTASE, ALTERNATIVE PATHWAY).Complement Factor B: A glycine-rich, heat-labile serum glycoprotein that contains a component of the C3 CONVERTASE ALTERNATE PATHWAY (C3bBb). Bb, a serine protease, is generated when factor B is cleaved by COMPLEMENT FACTOR D into Ba and Bb.Complement Factor D: A serum protein which is important in the ALTERNATIVE COMPLEMENT ACTIVATION PATHWAY. This enzyme cleaves the COMPLEMENT C3B-bound COMPLEMENT FACTOR B to form C3bBb which is ALTERNATIVE PATHWAY C3 CONVERTASE.Databases, Protein: Databases containing information about PROTEINS such as AMINO ACID SEQUENCE; PROTEIN CONFORMATION; and other properties.Internet: A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.User-Computer Interface: The portion of an interactive computer program that issues messages to and receives commands from a user.Software: Sequential operating programs and data which instruct the functioning of a digital computer.Proteins: Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.Sequence Analysis, Protein: A process that includes the determination of AMINO ACID SEQUENCE of a protein (or peptide, oligopeptide or peptide fragment) and the information analysis of the sequence.Glomerulonephritis, Membranoproliferative: Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS, increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. This may appear as a primary disorder or secondary to other diseases including infections and autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Various subtypes are classified by their abnormal ultrastructures and immune deposits. Hypocomplementemia is a characteristic feature of all types of MPGN.Hemolytic-Uremic Syndrome: A syndrome that is associated with microvascular diseases of the KIDNEY, such as RENAL CORTICAL NECROSIS. It is characterized by hemolytic anemia (ANEMIA, HEMOLYTIC); THROMBOCYTOPENIA; and ACUTE RENAL FAILURE.Complement Activation: The sequential activation of serum COMPLEMENT PROTEINS to create the COMPLEMENT MEMBRANE ATTACK COMPLEX. Factors initiating complement activation include ANTIGEN-ANTIBODY COMPLEXES, microbial ANTIGENS, or cell surface POLYSACCHARIDES.Complement C3: A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase.Complement C3b Inactivator Proteins: Endogenous proteins that inhibit or inactivate COMPLEMENT C3B. They include COMPLEMENT FACTOR H and COMPLEMENT FACTOR I (C3b/C4b inactivator). They cleave or promote the cleavage of C3b into inactive fragments, and thus are important in the down-regulation of COMPLEMENT ACTIVATION and its cytolytic sequence.Heparin: A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts.Liver Cirrhosis, Experimental: Experimentally induced chronic injuries to the parenchymal cells in the liver to achieve a model for LIVER CIRRHOSIS.Factor V Deficiency: A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren's disease or parahemophilia. It varies greatly in severity. Factor V deficiency is an autosomal recessive trait. (Dorland, 27th ed)Autistic Disorder: A disorder beginning in childhood. It is marked by the presence of markedly abnormal or impaired development in social interaction and communication and a markedly restricted repertoire of activity and interest. Manifestations of the disorder vary greatly depending on the developmental level and chronological age of the individual. (DSM-V)Hemophilia A: The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.Factor VIII: Blood-coagulation factor VIII. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation and this action is markedly enhanced by small amounts of thrombin.Genome: The genetic complement of an organism, including all of its GENES, as represented in its DNA, or in some cases, its RNA.Ecchymosis: Extravasation of blood into the skin, resulting in a nonelevated, rounded or irregular, blue or purplish patch, larger than a petechia.Complement Hemolytic Activity Assay: A screening assay for circulating COMPLEMENT PROTEINS. Diluted SERUM samples are added to antibody-coated ERYTHROCYTES and the percentage of cell lysis is measured. The values are expressed by the so called CH50, in HEMOLYTIC COMPLEMENT units per milliliter, which is the dilution of serum required to lyse 50 percent of the erythrocytes in the assay.Complement Pathway, Alternative: Complement activation initiated by the interaction of microbial ANTIGENS with COMPLEMENT C3B. When COMPLEMENT FACTOR B binds to the membrane-bound C3b, COMPLEMENT FACTOR D cleaves it to form alternative C3 CONVERTASE (C3BBB) which, stabilized by COMPLEMENT FACTOR P, is able to cleave multiple COMPLEMENT C3 to form alternative C5 CONVERTASE (C3BBB3B) leading to cleavage of COMPLEMENT C5 and the assembly of COMPLEMENT MEMBRANE ATTACK COMPLEX.Authorship: The profession of writing. Also the identity of the writer as the creator of a literary production.Knowledge Bases: Collections of facts, assumptions, beliefs, and heuristics that are used in combination with databases to achieve desired results, such as a diagnosis, an interpretation, or a solution to a problem (From McGraw Hill Dictionary of Scientific and Technical Terms, 6th ed).Factor Analysis, Statistical: A set of statistical methods for analyzing the correlations among several variables in order to estimate the number of fundamental dimensions that underlie the observed data and to describe and measure those dimensions. It is used frequently in the development of scoring systems for rating scales and questionnaires.Electrophoresis, Capillary: A highly-sensitive (in the picomolar range, which is 10,000-fold more sensitive than conventional electrophoresis) and efficient technique that allows separation of PROTEINS; NUCLEIC ACIDS; and CARBOHYDRATES. (Segen, Dictionary of Modern Medicine, 1992)Patient Access to Records: The freedom of patients to review their own medical, genetic, or other health-related records.Adipose Tissue: Specialized connective tissue composed of fat cells (ADIPOCYTES). It is the site of stored FATS, usually in the form of TRIGLYCERIDES. In mammals, there are two types of adipose tissue, the WHITE FAT and the BROWN FAT. Their relative distributions vary in different species with most adipose tissue being white.Subcutaneous Fat: Fatty tissue under the SKIN through out the body.Adipose Tissue, White: Fatty tissue composed of WHITE ADIPOCYTES and generally found directly under the skin (SUBCUTANEOUS FAT) and around the internal organs (ABDOMINAL FAT). It has less vascularization and less coloration than the BROWN FAT. White fat provides heat insulation, mechanical cushion, and source of energy.Obesity: A status with BODY WEIGHT that is grossly above the acceptable or desirable weight, usually due to accumulation of excess FATS in the body. The standards may vary with age, sex, genetic or cultural background. In the BODY MASS INDEX, a BMI greater than 30.0 kg/m2 is considered obese, and a BMI greater than 40.0 kg/m2 is considered morbidly obese (MORBID OBESITY).Complement C2: A component of the CLASSICAL COMPLEMENT PATHWAY. C2 is cleaved by activated COMPLEMENT C1S into COMPLEMENT C2B and COMPLEMENT C2A. C2a, the COOH-terminal fragment containing a SERINE PROTEASE, combines with COMPLEMENT C4B to form C4b2a (CLASSICAL PATHWAY C3 CONVERTASE) and subsequent C4b2a3b (CLASSICAL PATHWAY C5 CONVERTASE).Antigens, CD55: GPI-linked membrane proteins broadly distributed among hematopoietic and non-hematopoietic cells. CD55 prevents the assembly of C3 CONVERTASE or accelerates the disassembly of preformed convertase, thus blocking the formation of the membrane attack complex.Antigens, CD59: Small glycoproteins found on both hematopoietic and non-hematopoietic cells. CD59 restricts the cytolytic activity of homologous complement by binding to C8 and C9 and blocking the assembly of the membrane attack complex. (From Barclay et al., The Leukocyte Antigen FactsBook, 1993, p234)Complement System Proteins: Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY).C-Reactive Protein: A plasma protein that circulates in increased amounts during inflammation and after tissue damage.Endothelial Cells: Highly specialized EPITHELIAL CELLS that line the HEART; BLOOD VESSELS; and lymph vessels, forming the ENDOTHELIUM. They are polygonal in shape and joined together by TIGHT JUNCTIONS. The tight junctions allow for variable permeability to specific macromolecules that are transported across the endothelial layer.Complement Inactivator Proteins: Serum proteins that negatively regulate the cascade process of COMPLEMENT ACTIVATION. Uncontrolled complement activation and resulting cell lysis is potentially dangerous for the host. The complement system is tightly regulated by inactivators that accelerate the decay of intermediates and certain cell surface receptors.Juglans: A plant genus of the family JUGLANDACEAE that provides the familiar walnut.Complement C5: C5 plays a central role in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C5 is cleaved by C5 CONVERTASE into COMPLEMENT C5A and COMPLEMENT C5B. The smaller fragment C5a is an ANAPHYLATOXIN and mediator of inflammatory process. The major fragment C5b binds to the membrane initiating the spontaneous assembly of the late complement components, C5-C9, into the MEMBRANE ATTACK COMPLEX.Insulin-Like Growth Factor I: A well-characterized basic peptide believed to be secreted by the liver and to circulate in the blood. It has growth-regulating, insulin-like, and mitogenic activities. This growth factor has a major, but not absolute, dependence on GROWTH HORMONE. It is believed to be mainly active in adults in contrast to INSULIN-LIKE GROWTH FACTOR II, which is a major fetal growth factor.Neisseria meningitidis: A species of gram-negative, aerobic BACTERIA. It is a commensal and pathogen only of humans, and can be carried asymptomatically in the NASOPHARYNX. When found in cerebrospinal fluid it is the causative agent of cerebrospinal meningitis (MENINGITIS, MENINGOCOCCAL). It is also found in venereal discharges and blood. There are at least 13 serogroups based on antigenic differences in the capsular polysaccharides; the ones causing most meningitis infections being A, B, C, Y, and W-135. Each serogroup can be further classified by serotype, serosubtype, and immunotype.Neisseria: A genus of gram-negative, aerobic, coccoid bacteria whose organisms are part of the normal flora of the oropharynx, nasopharynx, and genitourinary tract. Some species are primary pathogens for humans.Staphylococcus epidermidis: A species of STAPHYLOCOCCUS that is a spherical, non-motile, gram-positive, chemoorganotrophic, facultative anaerobe. Mainly found on the skin and mucous membrane of warm-blooded animals, it can be primary pathogen or secondary invader.Tumor Necrosis Factor-alpha: Serum glycoprotein produced by activated MACROPHAGES and other mammalian MONONUCLEAR LEUKOCYTES. It has necrotizing activity against tumor cell lines and increases ability to reject tumor transplants. Also known as TNF-alpha, it is only 30% homologous to TNF-beta (LYMPHOTOXIN), but they share TNF RECEPTORS.Staphylococcus aureus: Potentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.Bacteremia: The presence of viable bacteria circulating in the blood. Fever, chills, tachycardia, and tachypnea are common acute manifestations of bacteremia. The majority of cases are seen in already hospitalized patients, most of whom have underlying diseases or procedures which render their bloodstreams susceptible to invasion.Escherichia coli: A species of gram-negative, facultatively anaerobic, rod-shaped bacteria (GRAM-NEGATIVE FACULTATIVELY ANAEROBIC RODS) commonly found in the lower part of the intestine of warm-blooded animals. It is usually nonpathogenic, but some strains are known to produce DIARRHEA and pyogenic infections. Pathogenic strains (virotypes) are classified by their specific pathogenic mechanisms such as toxins (ENTEROTOXIGENIC ESCHERICHIA COLI), etc.Gram-Negative Bacterial Infections: Infections caused by bacteria that show up as pink (negative) when treated by the gram-staining method.

In vitro analysis of complement-dependent HIV-1 cell infection using a model system. (1/80)

Previous studies based on the use of human serum as a source of C have provided evidence for the C-dependent enhancement of cell infection by HIV-1. The present study was undertaken to distinguish C from other serum factors and to identify the proteins and the mechanisms involved in C-dependent cell infection by HIV-1. The classical C activation pathway was reconstituted from the proteins C1q, C1r, C1s, C4, C2, C3, factor H, and factor I; each were purified to homogeneity. A mixture of these proteins at physiological concentrations was shown to reproduce the ability of normal human serum to enhance the infection of MT2 cells by HIV-1 at low doses of virus. This enhancing effect was abolished when heat-inactivated serum and C2- or C3-depleted serum were used, and was restored upon addition of the corresponding purified proteins. A mixture of two synthetic peptides corresponding to positions 10-15 and 90-97 of human C receptor type 2 (CD21) as well as soluble CD4 both inhibited the C-dependent infection process. These data provide unambiguous evidence that HIV-1 triggers a direct activation of the classical C pathway in vitro and thereby facilitates the infection of MT2 cells at low doses of virus. These findings are consistent with a mechanism involving increased interaction between the virus opsonized by C3b-derived fragment(s) and the CD21 cell receptors and subsequent virus entry through CD4 receptors.  (+info)

A cluster of positively charged amino acids in the C4BP alpha-chain is crucial for C4b binding and factor I cofactor function. (2/80)

C4b-binding protein (C4BP) is a regulator of the classical complement pathway, acting as a cofactor to factor I in the degradation of C4b. Computer modeling and structural analysis predicted a cluster of positively charged amino acids at the interface between complement control protein modules 1 and 2 of the C4BP alpha-chain to be involved in C4b binding. Three C4BP mutants, R39Q, R64Q/R66Q, and R39Q/R64Q/R66Q, were expressed and assayed for their ability to bind C4b and to function as factor I cofactors. The apparent affinities of R39Q, R64Q/R66Q, and R39Q/R64Q/R66Q for immobilized C4b were 15-, 50-, and 140-fold lower, respectively, than that of recombinant wild type C4BP. The C4b binding site demonstrated herein was also found to be a specific heparin binding site. In C4b degradation, the mutants demonstrated decreased ability to serve as factor I cofactors. In particular, the R39Q/R64Q/R66Q mutant was inefficient as cofactor for cleavage of the Arg937-Thr938 peptide bond in C4b. In contrast, the factor I mediated cleavage of Arg1317-Asn1318 bond was less affected by the C4BP mutations. In conclusion, we identify a cluster of amino acids that is part of a C4b binding site involved in the regulation of the complement system.  (+info)

Molecular cloning, expression and characterization of the rat analogue of human membrane cofactor protein (MCP/CD46). (3/80)

In humans, host cells are protected from homologous complement by membrane proteins encoded in the regulators of complement activation (RCA) gene cluster. These include complement receptor 1 (CR1), decay-accelerating factor (DAF, CD55) and membrane cofactor protein (MCP, CD46). In mouse and rat a single membrane inhibitor, Crry, appeared to perform the functions of both DAF and MCP and was proposed to be the functional analogue of both. Recently, however, murine homologues of DAF and MCP have been identified, prompting a search for the rat counterparts. We have described the identification of rat DAF and here describe the cloning of rat MCP from cDNA and genomic libraries, using a probe based on the mouse MCP cDNA sequence. The domain structure for rat MCP was identical to that of mouse MCP with four short consensus repeats (SCRs) followed by a STP domain, transmembrane segment and cytoplasmic tail. Overall identity of rat and mouse MCP was 77% at the amino acid level and 88% at the nucleotide level. Northern blot analysis from a range of tissues indicated that high-level expression was limited to the testis, although expression in other tissues was detected using reverse transcription-polymerase chain reaction. Rat MCP mRNA localized to Sertoli cells and spermatogonia in seminiferous tubules by in situ hybridization, but was absent in mature sperm. In cofactor assays utilizing human factor I, a recombinant soluble form of rat MCP catalysed cleavage of human C3ma.  (+info)

Function of the factor I modules (FIMS) of human complement component C6. (4/80)

In order to elucidate the function of complement component C6, truncated C6 molecules were expressed recombinantly. These were either deleted of the factor I modules (FIMs) (C6des-748-913) or both complement control protein (CCP) modules and FIMs (C6des-611-913). C6des-748-913 exhibited approximately 60-70% of the hemolytic activity of full-length C6 when assayed for Alternative Pathway activity, but when measured for the Classical Pathway, C6des-748-914 was only 4-6% as effective as C6. The activity difference between C6 and C6des-748-913 for the two complement pathways can be explained by a greater stability of newly formed metastable C5b* when produced by the Alternative Pathway compared with that made by the Classical Pathway. The half-lives of metastable C5b* and the decay of (125)I-C5b measured from cells used to activate the Alternative Pathway were found to be about 5-12-fold longer than those same parameters derived from cells that had activated the Classical Pathway. (125)I-C5 binds reversibly to C6 in an ionic strength-dependent fashion, but (125)I-C5 binds only weakly to C6des-FIMs and not at all to C6des-CCP/FIMs. Therefore, although the FIMs are not required absolutely for C6 activity, these modules promote interaction of C6 with C5 enabling a more efficient bimolecular coupling ultimately leading to the formation of the C5b-6 complex.  (+info)

Rat complement factor I: molecular cloning, sequencing and expression in tissues and isolated cells. (5/80)

Factor I (FI) is a regulatory serine protease of the complement system which cleaves three peptide bonds in the alpha-chain of C3b and two bonds in the alpha-chain of C4b thereby inactivating these proteins. The human protein and the recently characterized mouse factor I are heterodimers of about 88,000 MW which consist of a non-catalytic heavy chain of 50,000 MW which is linked to a catalytic light chain of 38,000 MW by a disulphide bond. For the screening of a rat liver cDNA library we used a hybridization probe produced by polymerase chain reaction (PCR) using degenerated primers which corresponded to conserved parts of the human and the murine factor I nucleotide sequences. One of the identified sequences, which had a length of 2243 base pairs (bp), contained the complete coding region and the whole 3' untranslated region. The length of the coding region in rat consisted of 1812 bp followed by a 3' untranslated region of 207 bp including the polyadenylation signal and the beginning of the poly A tail. Comparison of the rat cDNA-derived coding sequence revealed identities of 87% to the mouse and of 78% to the human FI nucleotide sequence. The translation product of rat FI mRNA was 604 amino acid residues (aa) in length with an identity of 85% to the mouse (603 aa) and 69% to the human protein (583 aa). The comparison of the molecular mass predicted by the primary structure and derived from rat FI isolated from rat serum as detected in immunoblot analyses suggested a glycosylation of more than 20% of the total mass of the FI protein. Expression studies using reverse transcription (RT)-PCR assays indicated that FI-specific mRNA could neither be identified in B cells, nor in T cells, monocytes or granulocytes from rat and human peripheral blood nor in rat peritoneal macrophages. These data were in agreement with the results of RT-PCR obtained with several human lymphoma cell lines (Jurkat, MOLT-4, HUT102, Wil 2-NS, Ramos, Raji, U937) all of which were devoid of FI-specific mRNA. In accord with our data from two rat hepatoma cell lines (FAO and H4IIE) and one from man (HepG2) only isolated rat hepatocytes (HC) but neither Kupffer cells (KC), hepatic stellate cells (HSC; Ito cells) nor sinusoidal endothelial cells (SEC) expressed FI-specific mRNA. FI mRNA was also detected in human umbilical vein endothelial cells (HUVEC) and in the uterus and small intestine of the rat. Spleen and lymph nodes did not contain any detectable FI-specific mRNA.  (+info)

Regulation of complement classical pathway by association of C4b-binding protein to the surfaces of SK-OV-3 and Caov-3 ovarian adenocarcinoma cells. (6/80)

The role of fluid-phase regulators of complement is to inhibit excessive complement activation and maintain homeostasis in blood. By binding to and inactivating complement components on cell surfaces, they can also protect autologous cells from complement-mediated cytotoxicity and phagocytosis. In this study, we wanted to find out whether C4b-binding protein (C4bp), a fluid-phase regulator of the classical complement pathway, could directly bind to cell surfaces in a functionally active form. After screening several malignant cell lines, we observed that the ovarian adenocarcinoma cell lines SK-OV-3, Caov-3, and SW626 were capable of binding C4bp. Binding tests with recombinant deletion mutants suggested that the primary binding site on C4bp is located on the alpha-chain complement control protein 4 domain. Functional tests showed that tumor cell-bound C4bp retained its cofactor activity for factor I-mediated inactivation of C4b, thus increasing the control of classical complement pathway activation on the surfaces of these cells. These results demonstrate a novel mechanism of complement regulation on cell surfaces, particularly on those of malignant ovarian tumor cells.  (+info)

Mapping of the sites responsible for factor I-cofactor activity for cleavage of C3b and C4b on human C4b-binding protein (C4bp) by deletion mutagenesis. (7/80)

Human C4b-binding protein (C4bp) facilitates the factor I-mediated proteolytic cleavage of the active forms of complement effectors C3b and C4b into their inactive forms. C4bp comprises a disulfide-linked heptamer of alpha-chains with complement (C) regulatory activity and a beta-chain. Each alpha-chain contains 8 short consensus repeat (SCR) domains. Using SCR-deletion mutants of recombinant multimeric C4bp, we identified the domains responsible for the C3b/C4b-binding and C3b/C4b-inactivating cofactor activity. The C4bp mutant with deletion of SCR2 lost the C4b-binding ability, as judged on C3b/C4b-Sepharose binding assaying and ELISA. In contrast, the essential domains for C3b-binding extended more to the C-terminus, exceeding SCR4. Using fluid phase cofactor assaying and deletion mutants of C4bp, SCR2 and 3 were found to be indispensable for C4b cleavage by factor I, and SCR1 contributed to full expression of the factor I-mediated C4b cleaving activity. On the other hand, SCR1, 2, 3, 4, and 5 participated in the factor I-cofactor activity for C3b cleavage, and SCR2, 3, and 4 were absolutely required for C3b inactivation. Thus, different sets of SCRs participate in C3b and C4b inactivation, and the domain repertoire supporting C3b cofactor activity is broader than that supporting C4b inactivation by C4bp and factor I. Furthermore, the domains participating in C3b/C4b binding are not always identical to those responsible for cofactor activity. The necessity of the wide range of SCRs in C3b inactivation compared to C4b inactivation by C4bp and factor I may reflect the physiological properties of C4bp, which is mainly directed to C4b rather than C3b.  (+info)

Human complement factor I does not require cofactors for cleavage of synthetic substrates. (8/80)

Complement factor I (fI) plays a major role in the regulation of the complement system. It circulates in an active form and has very restricted specificity, cleaving only C3b or C4b in the presence of a cofactor such as factor H (fH), complement receptor type 1, membrane cofactor protein, or C4-binding protein. Using peptide-7-amino-4-methylcoumarin derivatives, we investigated the substrate specificity of fI. There is no previous report of synthetic substrate cleavage by fI, but five substrates were found in this study. A survey of 15 substrates and a range of inhibitors showed that fI has specificity similar to that of thrombin, but with much lower catalytic activity than that of thrombin. fI amidolytic activity has a pH optimum of 8.25, typical of serine proteases and is insensitive to ionic strength. This is in contrast to its proteolytic activity within the fI-C3b-fH reaction, in which the pH optimum for C3b cleavage is <5.5 and the reaction rate is highly dependent on ionic strength. The rate of cleavage of tripeptide 7-amino-4-methylcoumarins by fI is unaffected by the presence of fH or C3(NH(3)). The amidolytic activity is inhibited by the synthetic thrombin inhibitor Z-D-Phe-Pro-methoxypropylboroglycinepinanediol ester, consistent with previous reports, and by benzenesulfonyl fluorides such as Pefabloc SC. Suramin inhibits fI directly at concentration of 1 mM. Within a range of metal ions tested, only Cr(2+) and Fe(3+) were found to inhibit both the proteolytic and amidolytic activity of fI.  (+info)

Anti-CFI / Complement Factor I Antibody (Internal), Rabbit Anti Human Polyclonal Antibody validated in WB, IHC-P (ALS17749), Abgent
Maus Monoklonal Complement Factor I Antikörper für ELISA, WB. Jetzt diesen anti-Complement Factor I Antikörper bestellen. | Produkt ABIN4264851
Complete information for CFI gene (Protein Coding), Complement Factor I, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
Factor I antibody [OX-21] (complement factor I) for ELISA, IP, RIA, WB. Anti-Factor I mAb (GTX41626) is tested in Human samples. 100% Ab-Assurance.
若限制補體活化的調節蛋白缺陷(ex. Complement H、Complement factor I、CD46等),會造成補體不正常的活化,造成血管內皮和腎臟細胞的傷害。此狀況可能為先天性,也可能是後天產生(產生自體抗體). ...
article{b6c22ab7-8314-43e4-a60f-8c80bb2fb02a, author = {Blom, Anna and Kask, Lena and Ramesh, Bala and Hillarp, Andreas}, issn = {0003-9861}, language = {eng}, number = {2}, pages = {108--118}, publisher = {Academic Press}, series = {Archives of Biochemistry and Biophysics}, title = {Effects of zinc on factor I cofactor activity of C4b-binding protein and factor H.}, url = {http://dx.doi.org/10.1016/j.abb.2003.08.018}, volume = {418}, year = {2003 ...
An introduction to information on haemophilia, von Willebrand Disorder and related bleeding disorders and related information on hepatitis C and HIV.
The protein encoded by this gene is similar to insulin in function and structure and is a member of a family of proteins involved in mediating growth and development. The encoded protein is processed from a precursor, bound by a specific receptor, and secreted. Defects in this gene are a cause of insulin-like growth factor I deficiency. Alternative splicing results in multiple transcript variants encoding different isoforms that may undergo similar processing to generate mature protein. [provided by RefSeq, Sep 2015 ...
Eculizumab is a monoclonal antibody that prevents complement activation. It has been found to be an effective treatment for atypical hemolytic-uremic syndrome (aHUS). This retrospective study is the largest collection of previously published and unpublished cases to date. Eculizumab was effective at both preventing and treating recurrence of aHUS.. ...
The purpose of this study is to determine whether eculizumab is safe and effective in the treatment of adult patients with plasma therapy-sensitive Atypical Hemolytic-Uremic Syndrome (aHUS).
This is a Phase 3, multicenter study of OMS721 in adults and adolescents with atypical hemolytic uremic syndrome (aHUS). The uncontrolled, open-label study will evaluate the effect of OMS721 in subjects with plasma therapy-resistant aHUS and plasma therapy-responsive aHUS. This study has four periods: Screening, Treatment Induction, Treatment Maintenance, and Follow-up. Approximate enrollment is 80 subjects. An interim analysis will be performed after 40 subjects have completed 26 weeks of treatment for potential registration ...
Abstract. Abstract 2085Introduction:. Atypical hemolytic uremic syndrome (aHUS) is a rare chronic disorder characterized by persistent uncontrolled complement
The primary purpose is to assess the efficacy and safety of eculizumab in pediatric patients with aHUS to control TMA as characterized by thrombocytopenia, hemolysis and renal impairment.
Zuber J, Le Quintrec M, Krid S, Bertoye C, Gueutin V, Lahoche A et al (2012) Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant 12(12):3337-3354. doi:10.1111/j.1600-6143.2012.04252.x CrossRefPubMedGoogle Scholar ...
All patients received open-label eculizumab administered intravenously on the following dose schedule: Induction dose - 900 mg per week for four weeks and a dose of 1200 mg one week later; Maintenance dose - 1200 mg every two weeks. Patients who received plasma exchange or infusion during the eculizumab treatment period received a supplemental dose of 600 mg within one hour before plasma infusion or within one hour after the completion of each plasma exchange ...
Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb tickover convertase. The actual occurrence of C3 glomerulopathy could be underestimated, since infection-related GN often quickly subsides without the need for a renal biopsy. A more thorough understanding of the pathways that lead to complement assembly and deposition within the kidney is needed to support a new classification of complement-related lesions, including entities such as dense deposit disease, (atypical) hemolytic-uremic syndrome, dominant C1q, CFHR5, C4d, and C3 ...
Information for healthcare professionals for diagnosing and treating Atypical Hemolytic Uremic Syndrome. Soliris is the only therapy approved for the treatment of aHUS.
Read about a case report study describing the clinical case of a patient with atypical hemolytic uremic syndrome (aHUS) associated with heart disease.
CHESHIRE, CT, Nov. 9, 2013- Soliris® (eculizumab) Inhibits TMA and Improves Renal Function in Pediatric and Adult Patients with atypical Hemolytic Uremic Syndrome (aHUS).
In a significant development the FDA in the USA approved Soliris (Eculizumab) for use in atypical Hemolytic Uremic Syndrome. To those saying,
For kidney fans, this review article on atypical hemolytic-uremic syndrome in this months New England Journal of Medicine (subscription required) is a must read. It details recent advances in the genetics of atypical hemolytic-uremic syndrome. What was previously a confusing mess of similar-appearing diseases - hemolytic-uremic syndrome, atypical hemolytic-uremic syndrome, drug-induced hemolytic-uremic syndrome, and thrombotic thrombocytopenic purpura - may finally be understandable ...
Atypical hemolytic uremic syndrome (aHUS) is an extremely rare, life-threatening, progressive disease that frequently has a genetic component. In most cases it is caused by chronic, uncontrolled activation of the complement system, a branch of the bodys immune system that destroys and removes foreign particles. The disease affects both children and adults and is characterized by systemic thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to stroke, heart attack, kidney failure, and death. The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane cofactor protein), or is occasionally due to acquired neutralizing autoantibody inhibitors of these complement system components, for example anti-factor H antibodies. Despite the use of supportive care, historically an estimated 33-40% of patients died or developed end-stage renal disease (ESRD) with the first ...
Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins. Thus, aHUS provides an archetypal complement-mediated disease with which to model new therapeutic strategies and treatments. Herein, we show that, when transferred to mice, an aHUS-associated gain-of-function change (D1115N) to the complement-activation protein C3 results in aHUS. Homozygous C3 p.D1115N (C3KI) mice developed spontaneous chronic thrombotic microangiopathy together with hematuria, thrombocytopenia, elevated creatinine, and evidence of hemolysis. Mice with active disease had reduced plasma C3 with C3 fragment and C9 deposition within the kidney. Therapeutic blockade or genetic deletion of C5, a protein downstream of C3 in the complement cascade, protected homozygous C3KI mice from thrombotic microangiopathy and aHUS. Thus, our data provide in vivo modeling evidence that ...
CONCLUSIONS: Morbidity rate is higher in children with aHUS. The renal prognosis and morbidity rate is higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS children with CFH mutation depends on the genetic background. PMID: 31705748 [PubMed - in process]...
Nineteen years after Gasser et al. [1] reported HUS, an interesting report was published in the Lancet [10]. This report indicated that although C3-predominant activity is initiated in the blood vessels in TMA patients, this is not observed in typical cases of HUS, suggesting that complement activation is involved in aHUS onset [12]. Subsequently, numerous researchers have elucidated further information on the pathology of aHUS. At present, the reported causes of aHUS include, complement regulation abnormalities, cobalamin metabolism disorder, infection with Streptococcus pneumoniae and other microorganisms, drugs, pregnancy, and autoimmune diseases.. The complement system plays an important role as part of the immune systems of living organisms. It is activated via 3 pathways, the classical, alternative, and lectin pathways. As a result of the activation of the hosts alternative and classical pathways, C5b-9, a membrane attack complex, is generated and destroys cells by forming transmembrane ...
Complement factor H (CFH) protein is an inhibitor of the alternative pathway of complement (AP) both in the fluid phase and on the surface of host cells. Mouse and human complement factor H-related (CFHR) proteins also belong to the fH family of plasma glycoproteins. The main goal of the current study was to compare the presence of mRNA for two mCFHR proteins in spontaneously developing autoimmune diseases in mice such as dense deposit disease (DDD), diabetes mellitus (DM), basal laminar deposits (BLD), collagen antibody-induced arthrits (CAIA) and systemic lupus erythematosus (SLE). Here we report for the first time that the CFHR-C mRNA was universally absent in the liver from three strains of lupus-prone mice and in a diabetic-prone mouse strain. The mRNA levels (pg/ng) for CFH and CFHR-B in MRL-lpr/lpr, at 9wks and 23wks were 707.2±44.4, 54.5±5.75 and 729±252.9, 74.04±22.76, respectively. The mRNA levels for CFH and CFHR-B in NZB/NZW mice, at 9wks and 54wks were 579.9±23.8, 58.8±1.41 ...
Alexions product Soliris® (eculizumab), is a therapy indicated for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH).
A diagnosis of thrombotic microangiopathy on kidney biopsy in a patient presenting with hypertensive emergency has historically elicited the diagnosis of malignant hypertension-associated thrombotic microangiopathy. Recent studies, however, have raised awareness that a number of these patients may actually represent atypical hemolytic uremic syndrome. To further investigate this premise, we performed next-generation sequencing to interrogate the coding regions of 29 complement and coagulation cascade genes associated with atypical hemolytic uremic syndrome in 100 non-elderly patients presenting with severe hypertension, renal failure and a kidney biopsy showing microangiopathic changes limited to the classic accelerated hypertension-associated lesion of arterial intimal edema (mucoid intimal hyperplasia) in isolation and without accompanying glomerular microthrombi ...
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A plea on behalf of CFI Canadas volunteers: please dont blame us for the crap that goes on at head office!. Payton and Trottier are completely removed from the work of the local branches. CFI Vancouver is an excellent organisation, as is CFI Ottawa (@Dick the Damned, Im also a Brit living in Ottawa and am heavily involved with the group so get in touch and Ill hook you up - it would be great to have you here, also see http://www.meetup.com/Ottawa-Skeptics/ :-) ), and I have heard great things about the branches in Montreal, Edmonton and Calgary.. These groups (representing the majority of CFI Canadas activity) are run by volunteers who pour their time and effort into making CFI great, and it is SO sad to see it undermined by those who are being paid from our memberships… CFI Canada is the only gig in town as far as Canadian skeptical organisations go, so its all we have! CFI Canada is not Payton. CFI Canada is the many volunteers who work so hard to advance reason and skepticism in ...
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On Monday 23 September from 10 am to 1 pm Centre for Internet Studies will host an open orientation meeting about CFI and the centres activities.
4AYI: Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type
14:52 - The punchline: aHUS and TTP diagnosis algorithm. Publications. Stromsness B. et al. Physician Interpretation of Equivocal Results for aHUS Genetic Testing Varies Greatly and is Frequently at Odds with Laboratory Views. J Clin Apheresis. 2019; (abstract P-82).. Ero, MP, Kain, JS, inventors; Machaon Diagnostics, Inc., assignee. 2018 Dec 18. Method of diagnosis of complement-mediated thrombotic microangiopathies. United States patent US 10,155,983.. Tao J. et al. A rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis. BMC Nephrology. 2018;19:355.. Kain J. et al. Additional Genes Associated with Atypical Hemolytic Uremic Syndrome. ASN 2018 Abstract TH-PO713. 2018; (abstract).. Switala L. et al. Complement factor abnormalities detected in patients with suspected Heparin-induced Thrombocytopenia (HIT) but not in Thrombotic Thrombocytopenia Purpura (TTP). ISLH 2017 Abstract Proceedings. 2017; (abstract).. Ipe T. et al. An extremely rare ...
Atypical hemolytic-uremic syndrome (aHUS) is a rare, potentially lethal systemic disorder, capable of affecting both adults and children, causing thrombotic microangiopathy (TMA) that leads to the formation of thrombus within small blood vessels with multiple organ failure. The pathogenesis of the aHUS is part of a sort of chronic and uncontrolled activation of the complement system by genetic mutation of some proteins usually responsible for its self-regulation. Today, the rapid diagnosis of the disease and the timely start of treatment with eculizumab, improve outcomes of renal failure, stroke and heart attack. Fabry disease is a rare tesaurismosis, X linked, due to the deficiency of the lysosomal enzyme alpha-galactosidase A, necessary for the physiological catabolism of glycosphingolipids. Multisystem clinical manifestations lead to a serious degenerative pathology. The diagnostic suspicion based on anamnesis and careful research of the symptoms and then confirmed by the enzymatic dosage of ...
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SR GROUP - Exporter, Importer, Manufacturer, Distributor, Supplier, Trading Company of Rat CFH(Complement Factor H) ELISA Kit based in Delhi, India
CONTROL AND ANALYSIS OF PARTICULATE MATTER BY MEMBRANE FILTRATION. This system has exhibited tadalafil stability in the sense of, bar a number of notable exceptions, surface temperature remaining within the bounds required for liquid water and so a significant biosphere. Based on whether the PCT level was monitored or not, we divided patients into regular group and PCT group. C4NeFs were not detected in tadalafil 20 mg 150 patients with another complement-mediated kidney disease, atypical hemolytic uremic syndrome. The absorption of hydrophobic drugs and nutrients from the intestine is principally determined by the tadalafil 20 mg canadian drug stores amount that can be dissolved by the endogenous fluids present in the gut. The MLE algorithm searches for the image that has the maximum probability to generate the projection data.. Processing of pain- and body-related verbal material in chronic pain patients: central and peripheral correlates. However, genotyping of the flanking sequences on 22q ...
The Center for Life at Newcastle upon Tyne, UK, is organizing a conference for patients and their family on atypical Hemolytic Uremic Syndrome on Sat...
Homo sapiens membrane cofactor protein (CD46, trophoblast-lymphocyte cross-reactive antigen) (MCP), transcript variant h, mRNA. (H00004179-R27) - Products - Abnova
Homo sapiens membrane cofactor protein (CD46, trophoblast-lymphocyte cross-reactive antigen) (MCP), transcript variant n, mRNA. (H00004179-R17) - Products - Abnova
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Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation in mice.
14:52 - The punchline: aHUS and TTP diagnosis algorithm. Publications. Stromsness B. et al. Physician Interpretation of Equivocal Results for aHUS Genetic Testing Varies Greatly and is Frequently at Odds with Laboratory Views. J Clin Apheresis. 2019; (abstract P-82).. Ero, MP, Kain, JS, inventors; Machaon Diagnostics, Inc., assignee. 2018 Dec 18. Method of diagnosis of complement-mediated thrombotic microangiopathies. United States patent US 10,155,983.. Tao J. et al. A rare case of Alport syndrome, atypical hemolytic uremic syndrome and Pauci-immune crescentic glomerulonephritis. BMC Nephrology. 2018;19:355.. Kain J. et al. Additional Genes Associated with Atypical Hemolytic Uremic Syndrome. ASN 2018 Abstract TH-PO713. 2018; (abstract).. Switala L. et al. Complement factor abnormalities detected in patients with suspected Heparin-induced Thrombocytopenia (HIT) but not in Thrombotic Thrombocytopenia Purpura (TTP). ISLH 2017 Abstract Proceedings. 2017; (abstract).. Ipe T. et al. An extremely rare ...
Hemolytic-uremic syndrome (HUS) consists of three main symptoms. Red blood cells are destroyed resulting in in anemia (microangiopathic hemolytic anemia), low platelet count occurs in the blood (thrombocytopenia) and accumulation of platelets and red blood cells (microthrombi) blocks certain blood vessels resulting in decrease blood flow to organs such as the kidneys, pancreas and brain. This may result in kidney failure. Hypertension is often severe. For atypical HUS diarrhea cannot be present. Normal levels of f actor H do not exclude the presence of a CFH mutation. ...
TY - JOUR. T1 - Acute Kidney Injury in Pregnancy. AU - Jim, Belinda. AU - Garovic, Vesna D. PY - 2017/7/1. Y1 - 2017/7/1. N2 - Summary: Pregnancy-related acute kidney injury (AKI) has declined in incidence in the last three decades, although it remains an important cause of maternal and fetal morbidity and mortality. Pregnancy-related causes of AKI such as preeclampsia, acute fatty liver of pregnancy, HELLP (Hemolysis, Elevated Liver function tests, Low Platelets) syndrome, and the thrombotic microangiopathies (thrombotic thrombocytopenic purpura, atypical hemolytic-uremic syndrome [HUS]) exhibit overlapping features and often present as diagnostic dilemmas. Differentiating among these conditions may be difficult or impossible based on clinical criteria only. In difficult and rare cases, a renal biopsy may need to be considered for the exact diagnosis and to facilitate appropriate treatment, but the risks and benefits need to be carefully weighed. The use of eculizumab for the treatment of ...
The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
The complement control protein (CCP) modules (also known as short consensus repeats) are defined by a consensus sequence within a stretch of about 60 amino acid residues. These modules have been identified more than 140 times in over 20 proteins, including 12 proteins of the complement system. The solution structure of the 16th CCP module from human complement factor H has been determined by a combination of 2-dimensional nuclear magnetic resonance spectroscopy and restrained simulated annealing. In all, 548 structurally important nuclear Overhauser enhancement cross-peaks were quantified as distance restraints and, together with 41 experimentally measured angle restraints, were incorporated into a simulated annealing protocol to determine a family of closely related structures that satisfied the experimental observations. The CCP structure is shown to be based on a beta-sandwich arrangement; one face made up of three beta-strands hydrogen-bonded to form a triple-stranded region at its centre ...
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Terragnosis have just added a campaign supplement for Sulphur, the 32mm sci-fi battle game. Follow the link here and download a free PDF of the rules. Check them out, give them a try and feed back to us any comments, queries or clarifications you might have. ...
Membrane cofactor protein (MCP) is a complement regulatory protein that is expressed on human cells and cell lines as two relatively broad species with Mr of 58,000-68,000 and 48,000-56,000. The structure of a previously reported cDNA clone indicated that MCP was a type 1 membrane glycoprotein and a member of the regulators of complement activation gene/protein cluster. However, it did not provide an explanation for the unusual phenotypic pattern of MCP. Therefore, in parallel with an analysis of the gene, additional cDNAs were cloned and characterized. Six different MCP cDNA classes were identified. All encode the same 5 untranslated signal peptide, four SCRs, transmembrane domain, and basic amino acid anchor. However, they differ in the length and composition of an extracellular serine/threonine/proline (STP)-rich area, a site of heavy O-glycosylation, and cytoplasmic tail. Analysis of the MCP gene demonstrated that the variation in cDNA structure was a result of alternative splicing. ...
This case report represents how eculizumab reversed neurologic impairment and improved GW786034 renal damage in severe atypical hemolytic uremic syndrome. hemolytic uremic syndrome eculizumab safely reverses neurologic impairment and eliminates the need for dialysis. The optimal duration of treatment with eculizumab remains to be determined. also causes a severe form of hemolytic uremic syndrome unrelated to Shiga or Shiga-like toxin-producing organisms.8 9 A minority of hemolytic uremic syndrome cases generally unrelated to Shiga/Shiga-like toxin or mutations with cardiac complications in 20% of cases are associated with a worse long-term survival. mutations are associated with a 10-year survival rate of only 40%-50% compared with cases of anti-CFH antibodies mutations and mutations which have a 10-year survival rate of around 80%-90%. Furthermore hereditary testing supplies the clinician with prognostic and predictive information regarding the disease GW786034 program treatment response and ...
Shantee Anaquod is finally getting the life-saving drug she needs.. On Tuesday afternoon (Nov. 21), the province approved funding for the 23-year-old UBC student to receive Soliris, a drug that treats the rare disease Atypical Hemolytic Uremic Syndrome (aHUS) and costs $750,000 per year.. This comes after a Nov. 20 announcement by B.C. Health Minister Adrian Dix that the province will now cover the costs of Soliris on a case-by-case basis.. Shantee was diagnosed with aHUS shortly after Thanksgiving, and has been undergoing dialysis and plasma exchanges in hospital ever since. The ultra rare blood disease - which affects between 100 and 150 Canadians -causes a protein to be built that attacks the bodys own healthy cells, leading to blood clotting and kidney failure.. Previously, Shantee was denied coverage for the drug by both the B.C. government and her extended medical, despite other provinces like Ontario and Quebec covering the costs.. "After this was brought to his attention, the Minister ...
This study explores the relationship between defined antigens of the C3 molecule and those surface structures that are involved in interaction with factors I and H. Methylamine treatment at pH 11 followed by neutralization converts C3 into a modified state in which the molecule is optimally susceptible to cleavage by factor I in the presence of factor H. The modified C3 is characterized by an antigenic profile with expression of antigens of the C3(N), C3(S), and C3(D) subsets. These antigenic properties closely mirror those of physiologically bound C3b, suggesting that modification of antigenic expression upon denaturation of C3 reflects a regulatory mechanism for I and H function. Immunochemical studies of the alkaline-denatured C3 suggested that factor H interacts with surfaces of C3 that are situated within the C3c fragment and that are defined by C3(SN) antigens, while factor I predominantly interacts with C3(SN) antigens associated with the C3d fragment and with C3(D) antigens hidden in ...
1HFD: Structures of native and complexed complement factor D: implications of the atypical His57 conformation and self-inhibitory loop in the regulation of specific serine protease activity.
SEA635Mu, CF-H; FH; FHL1; ARMD4; ARMS1; CFHL3; HF1; HF2; HUS; H Factor 2; Age-Related Maculopathy Susceptibility 1; Adrenomedullin binding protein | Products for research use only!
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BFA CEO Dr Emídio Pinheiro (52) was born in Lisbon and obtained a degree in economics at Universidade Católica de Lisboa. He pursued his studies at the
Background: Vascular endothelial cells (ECs) express and release protein components of the complement pathways, as well as secreting and anchoring ultra-large von Willebrand factor (ULVWF) multimers in long string-like structures that initiate platelet adhesion during hemostasis and thrombosis. The alternative complement pathway (AP) is an important nonantibody- requiring host defense system. Thrombotic microangiopathies can be associated with defective regulation of the AP (atypical hemolytic-uremic syndrome) or with inadequate cleavage by ADAMTS-13 of ULVWF multimeric strings secreted by/anchored to ECs (thrombotic thrombocytopenic purpura). Our goal was to determine if EC-anchored ULVWF strings caused the assembly and activation of AP components, thereby linking two essential defense mechanisms. Methodology/Principal Findings: We quantified gene expression of these complement components in cultured human umbilical vein endothelial cells (HUVECs) by real-time PCR: C3 and C5; complement factor ...
Complement component C3 plays a central role in the activation of complement system. Its activation is required for both classical and alternative complement activation pathways. The encoded preproprotein is proteolytically processed to generate alpha and beta subunits that form the mature protein, which is then further processed to generate numerous peptide products. The C3a peptide, also known as the C3a anaphylatoxin, modulates inflammation and possesses antimicrobial activity. Mutations in this gene are associated with atypical hemolytic uremic syndrome and age-related macular degeneration in human patients. [provided by RefSeq, Nov 2015 ...
Mark, L, Spiller, OB, Okroj, M, Chanas, S, Aitken, JA, Wong, SW, Damania, B, Blom, AM and Blackbourn, DJ (2007) Molecular characterization of the rhesus rhadinovirus (RRV) ORF4 gene and the RRV complement control protein it encodes ...
By Brenden Schild In Portfolio Louisville, Kentucky - August 21st, 2013 - Rock Spring Ventures has increased its investment in Apellis Pharmaceuticals Inc., an early-stage biotechnology company focused on developing a novel pipeline of anti-inflammatory products through a combination of in-licensing of preclinical compounds and in-house research and development.. Apellis aims to bring a new class of anti-inflammatory drugs to the market to address major diseases by exploring novel mechanisms to modify these diseases by inhibiting complement activation. Apellis currently has two programs centered on the use of APL-1 in extra-ocular indications. APL-1 is a derivative of the cyclic peptide Compstatin, discovered at the University of Pennsylvania. APL-1 is a small cyclic peptide that binds to human complement factor C3 and prevents its activation, resulting in broad and potent complement activation inhibition. APL-1 is also known as POT-4, originally developed by Potentia Pharmaceuticals.. As an ...
Fremeaux-Bacchi, V.; Kemp, E. J.; Goodship, J. A.; Dragon-Durey, M. A.; Strain, L.; Loirat, C.; Deng, H. W.; Goodship, T. H. J. The development of atypical haemolytic-uraemic syndrome is influenced by susceptibility factors in factor H and membrane cofactor protein: evidence from two independent cohorts. Journal of medical genetics. 2005, NOV. 42(11):852-856 ...
Quantity100 testsVolume0.4ImmunogenHuman Acute Lymphocytic Leukemia (ALL) T cellsBackground InformationCD46 (MCP; membrane cofactor protein) is a m...
The Pediatric infectious disease journal; VOL: 11 (3); p. 203-9 Coutsoudis A; Kiepiela P; Coovadia HM; Broughton M. The effect of vitamin A supplementation on selected factors of immunity was tested in African children (ages 4 to 24 months with complicated measles) during a randomized double-blind intervention trial. Placebo (n = 31) and treated groups (n = 29) had similar baseline characteristics. The supplemented group had significant reductions in morbidity (expressed as integrated morbidity scores) during the acute (Day 8, P = 0.006) and chronic (Day 42, P = 0.02; 6 months; P = 0.002) phases. In the treated group there was an increase in total number of lymphocytes (Day 42, P = 0.05) and measles IgG antibody concentrations (Day 8, P = 0.02), both of which have consistently been previously shown to correlate more closely with outcome in measles than other immunologic, clinical and radiologic factors. Interleukin 2 and plasma complement values were unaffected by vitamin A supplementation. ...
Perehdy, onko ruuan lisäaine Kinoliinikeltainen (E104) ja muut elintarvikkeiden E-koodit terveydellesi haitaksi vai hyödyksi E-koodit.fi:stä.
Membrane cofactor protein (MCP), a regulatory molecular of the complement system with cofactor activity for the factor I-mediated inactivation of C3b and C4b, is widely distributed, being present on leukocytes, platelets, endothelial cells, epithelial cells, and fibroblasts. MCP was purified from a human T cell line (HSB2) and the NH2-terminal 24-amino acid sequence obtained by Edman degradation. An oligonucleotide probe based on this sequence was used to identify a clone from a human monocytic (U937) cDNA library. Nucleotide sequencing showed a 43-bp 5-untranslated region, an open reading frame of 1,152 bp, and a 335-bp 3-untranslated region followed by a 16-bp poly(A) track. The deduced full-length MCP protein consists of a 34-amino acid signal peptide and a 350-amino acid mature protein. The protein has, beginning at the NH2 terminus, four approximately 60-amino acid repeat units that match the consensus sequence found in a multigene family of complement regulatory proteins (C3b-receptor or ...
Pre- and postnatal growth failure can be caused by mutations in the IGF1 (insulin-like growth factor 1) or IGF1R (insulin-like growth factor 1 receptor) genes. Autosomal recessive mutations in IGF1 cause insulin-like growth factor I deficiency (MIM 608747), which is characterized by microcephaly, intellectual disability, and deafness in addition to the growth failure. Autosomal dominant mutations in IGF1R cause insulin-like growth factor I, resistance to (MIM 270450). It is associated with partial IGF1 resistance, pre- and postnatal growth failure, microcephaly, and in some cases modest intellectual disability. Autosomal recessive IGF1R mutations have been found in a few patients. This results in a more severe growth delay and additional developmental abnormalities.. Read less ...
Purpose of Review: Over the past two decades, significant strides made in our understanding of the etiology of antibody-mediated rejection (AMR) in transplantation have put the complement system in the spotlight. Here, we review recent progress made in the field of pharmacologic complement inhibition in clinical transplantation and aim to understand the impact of this therapeutic approach on outcomes in transplant recipients. Recent Findings: Encouraged by the success of agents targeting the complement cascade in disorders of unrestrained complement activation like paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS), investigators are testing the safety and efficacy of pharmacologic complement blockade in mitigating allograft injury in conditions ranging from AMR to recurrent post-transplant aHUS, C3 glomerulopathies and antiphospholipid anti-body syndrome (APS ...
Dr. Braunsteins research focuses on inherited predisposition to hematologic diseases. His laboratory studies the inherited genetic changes in DNA that increase susceptibility to disease. Blood cancers such as myeloproliferative neoplasms and myelodysplastic syndromes are traditionally thought to be acquired disorders, however there is increasing evidence that inherited genetic changes play a role. In addition, Dr. Braunstein studies non-malignant blood diseases including atypical hemolytic uremic syndrome (aHUS) and related thrombotic disorders such as APLS, TTP and HELLP syndrome which are caused in part by genetic mutations. His work has identified a germline variants in the ERBB genes that predispose to hematologic malignancies. In addition, his research group found that patients with catastrophic APLS and HELLP syndrome frequently harbor germline mutations in complement regulatory genes. This has led directly to clinical trials designed to test the efficacy of complement inhibitio...n in ...
Expertise, Disease and Conditions: Atherosclerotic Renovascular Disease, Atypical Hemolytic Uremic Syndrome (aHUS), Electrolyte Disorders, Fibromuscular Dysplasia (FMD), Glomerulonephritis, Hyperaldosteronism, Hypertension, Nephrology, Renal Artery ...
This collaborative 3-year research grant was awarded to young investigators Dr. Yongzhi Qiu and Dr. Satheesh Chonat under the mentorship of Dr. Wilbur Lam. Using a novel `microvasculature-on-a-chip technology, these Aflac researchers, along with Dr. Larry Greenbaum (Emory University), Dr. Traci Leong (Emory University) and Dr. Richard Smith (University of Iowa), will examine small blood clots are formed as a result of damage to the endothelium. The knowledge gained will advance understanding of atypical hemolytic uremic syndrome (aHUS), a rare genetic disorder that causes anemia, low platelet count and kidney damage ...
Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent studies have identified a factor H-associated form of HUS, caused by gene mutations that cluster in the C-terminal region of the complement regulator factor H. Here we report how three mutations (E1172Stop, R1210C, and R1215G; each of the latter two identified in three independent cases from different, unrelated families) affect protein function. All three mutations cause reduced binding to the central complement component C3b/C3d to heparin, as well as to endothelial cells. These defective features of the mutant factor H proteins explain progression of endothelial cell and microvascular damage in factor H-associated genetic HUS and indicate a protective role of factor H for tissue integrity during thrombus formation.. ...
Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent studies have identified a factor H-associated form of HUS, caused by gene mutations that cluster in the C-terminal region of the complement regulator factor H. Here we report how three mutations (E1172Stop, R1210C, and R1215G; each of the latter two identified in three independent cases from different, unrelated families) affect protein function. All three mutations cause reduced binding to the central complement component C3b/C3d to heparin, as well as to endothelial cells. These defective features of the mutant factor H proteins explain progression of endothelial cell and microvascular damage in factor H-associated genetic HUS and indicate a protective role of factor H for tissue integrity during thrombus formation.. ...
There is increasing evidence that human complement factor H-related protein 1 (CFHR1) plays a crucial role in the development of malignant diseases. However, few studies have identified the roles of CFHR1 in the occurrence and prognosis of lung adenocarcinoma (LADC). In the present study, comprehensive bioinformatic analyses of data obtained from the Oncomine platform, UALCAN and Gene Expression Profiling Interactive Analysis (GEPIA) demonstrated that CFHR1 expression is significantly reduced in both LADC tissues and cancer cells. The patients presenting with downregulation of CFHR1 had significantly lower overall survival (OS) and post progression survival (PPS) times. Through analysis of the datasets from Gene Expression Omnibus database, we found that the compound actinomycin D promoted CFHR1 expression, further displaying the cytotoxic effect in the LADC cell line A549. In addition, the expression level of CFHR1 in the cisplatin-resistant LADC cell line CDDP-R (derived from H460) was also ...
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BACKGROUND: Titin is a huge protein ( approximately 3 MDa) that is present in the contractile unit (sarcomere) of striated muscle and has a key role in muscle assembly and elasticity. Titin is mainly composed of two types of module (type I and II). Type I modules are found exclusively in the region of titin localised in the A band, where they are arranged in a super-repeat pattern that correlates with the ultrastructure of the thick filament. No structure of a titin type I module has been reported so far. RESULTS: We have determined the structure of a representative type I module, A71, using nuclear magnetic resonance (NMR) spectroscopy. The structure has the predicted fibronectin type III fold. Titin-specific conserved residues are either located at the putative module-module interfaces or along one side of the protein surface. Several proline residues that contribute to two stretches in a polyproline II helix conformation are solvent-exposed and line up as a continuous ribbon extending over ...
CFI is involved in poultry, milling of stockfeeds, maize and flour, manufacture and distribution of agri-chemicals, farming and retail. The environmentally-controlled houses will have capacity to keep 160 000 birds per six-week cycle. The current housing facilities are open-sided with capacity of about 460,000 birds ...
Center for Inquiry | Michigan (CFI) provides a community for atheists, humanists, agnostics, skeptics, and other freethinkers to explore ideas from a non-theistic perspective.
That proves it right there! I knew old Bill was sparkin a fatty when he wrote the "Tempest" and "A Midsummer Nights Dream". I had to read them for a Shakespeare class in college and never could, no wait a minute, oh yeah, I was sparkin the fatty while READING the plays. And Im telling ya that was some really good shit! Thanks Will for being the stoner Bard and inventing all of those cool words like Oclock. ...
β重型海洋性貧血4一般所說的重症海洋性貧血就是指「β重型海洋性貧血「3是β血紅蛋白鏈合成嚴重的不足》剛出生時3患有β重型海洋性貧血的寶寶在外觀上跟正常者沒有任何差別》但是漸漸地到了三到六個月大時3這時候因為β血紅蛋白鏈不夠3沒有辦法合成「成人血紅素「情況產生3因此會有臉色蒼白 食慾 活力變差...等貧血的症狀產生》一旦寶寶發病3後果嚴重3必須每隔二至三週輸血一次》長期的輸血會造成體內鐵質的沉積3導致體內器官逐漸喪失功能3最後常因心臟衰竭而在孩童時即死亡》另一方面3由於長期輸血3容易引起病毒的感染3例如B型 C型肝炎3和愛滋病等》真正要根治這種疾病3需要骨髓移植》我國目前在骨髓移植的成功率大約在60%3其餘40%的失敗者可能因併發症死亡3或者回復原來長期輸血打排鐵劑的狀況》 ...
第一卷(共115分)第一部分听力(共两节,满分30分)第一节(共5小题;每小题1.5分,满分7.5分)听下面5段对话。每段对话后有一个小题,从题中所给的A、B、C三个选项中选出最佳选项,并标在试卷的相应位置。听完每段对话后,你都有10秒钟的时间来回答有关小题和阅读下一小题。每段对话仅读一遍
This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. This protein co-localizes with C3, binds C3b in a dose-dependent manner, and is recruited to tissues damaged by C-reactive protein. Allelic variations in this gene have been associated, but not causally linked, with two different forms of kidney disease: membranoproliferative glomerulonephritis type II (MPGNII) and hemolytic uraemic syndrome (HUS). [provided by RefSeq, Jan 2010 ...
Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). C3 nephritic factors (C3NeF) play an important role in C3G pathogenesis by stabilizing the key enzymatic complex of complement, the C3 convertase. However, the reliability of assays detecting these autoantibodies is limited. Therefore, in this study, we validated and optimized a prototype hemolytic method for robust detection and characterization of factors causing convertase overactivity in large patient cohorts. The assay assesses convertase activity directly in the physiological milieu of serum and therefore is not restricted to detection of stabilizing autoantibodies such as C3NeF but may also reveal genetic variants resulting in prolonged convertase activity. We first defined clear cutoff values based on convertase activity in healthy controls. Next, we evaluated 27 C3G patient samples and found 16 positive for prolonged
From NCBI Gene:. This gene belongs to a family of complement factor H-related genes (CFHR), which are clustered together with complement factor H gene on chromosome 1, and are involved in regulation of complement. Mutations in CFHR genes have been associated with dense deposit disease and atypical haemolytic-uraemic syndrome. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2015]. From UniProt: ...
Dr. Arshad Ali (AA), from Emory University School of Medicine in Atlanta, Georgia, discusses his abstract for the National Kidney Foundations 2015 Spring Clinical Meetings (SCM15), Proliferative C4 Dense Deposition Disease Concurrent with Acute Thrombotic Microangiopathy in an Adult Patient with Acute Renal Failure, with Dr. Kenar Jhaveri, AJKD Blog Editor. AJKDblog: Why dont you…
The SCOP classification for the Complement control module/SCR domain family. Additional information, provided for both this family and the superfamily it belongs to, includes SUPERFAMILY links to genome assignments, alignments, domain combinations, taxonomic visualisation and hidden Markov model information.
aHUS patients raised the question " are the predisposing genetic factors of aHUS fully catalogued?" as a topic of research which matters to them in their Global Research Agenda.. Those affected by aHUS know well that it is imperfections in components of the Complement System that made them susceptible to the disease when one of many "triggering hits" over their lives caused a catastrophic onset of aHUS.. They know that there are different imperfections in different aHUS patients, some not yet found. But how many and who is keeping a record of what to look for as an aHUS "susceptibility imperfection" , or "mutation" or "significant variant".. At University College London, the Department of Structural and Molecular Biology has been collating variants in the Complement System for 15 years and creating a database of information about them. It is known as the Database of Complement Gene Variants and can seen online , click here.. Designed for use by scientist and clinicians the information held is ...
Hypothetical LOC387715 is a second major susceptibility gene for age-related macular degeneration, contributing independently of complement factor H to disease risk. ...
Rare Disease Day is 28 February 2018. The aHUS Alliance has announced a global project to raise awareness about the rare disease atypical HUS (aHUS).
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... factor H, factor HR1 or HR3, membrane cofactor protein, factor I, factor B, complement C3, and thrombomodulin). This results in ... The complement system activation may be due to mutations in the complement regulatory proteins (factor H, factor I, or membrane ... of complement can result from production of anti-factor H autoantibodies or from genetic mutations in any of several complement ... "Platelet-associated complement factor H in healthy persons and patients with atypical HUS". Blood. 114 (20): 4538-4545. doi: ...
PSAP Complement component 4, partial deficiency of; 120790; C1NH Complement factor H deficiency; 609814; HF1 Complement factor ... GLA Factor V and factor VIII, combined deficiency of; 227300; MCFD2 Factor V deficiency; 227400; F5 Factor XI deficiency, ... F11 Factor XII deficiency; 234000; F12 Factor XIIIA deficiency; 613225; F13A1 Factor XIIIB deficiency; 613235; F13B Failure of ... LCAT Fletcher factor deficiency; 612423; KLKB1 Focal cortical dysplasia, Taylor balloon cell type; 607341; TSC1 Focal dermal ...
Porins are also recognized by TLR2, they bind complement factors (C3b, C4b, factor H, and C4bp (complement factor 4b-binding ... Complement inhibition[edit]. Factor H binding protein (fHbp) that is exhibited in N. meningitidis and some commensal species is ... Close contact with a carrier is the predominant risk factor. Other risk factors include a weakened general or local immune ... Porins are also an important factor for complement inhibition for both pathogenic and commensal species. Porins are important ...
It binds to complement factor D (CFD). Enrollment in Phase 3 clinical trials was initiated in 2014. Phase 3 trials are usually ... These two failures have called into question whether complement inhibition is a sound strategy for geographic atrophy. World ...
"Onchocerca volvulus microfilariae avoid complement attack by direct binding of factor H". The Journal of Infectious Diseases. ... The complement system is used to enhance the effect of antibodies and phagocytic cells, which engulf and destroy other cells. ...
Some HLA alleles have been suspected along with complement phenotypes as being genetic factors. Non-aggressive Berger's disease ... Other blood tests done to aid in the diagnosis include CRP or ESR, complement levels, ANA, and LDH. Protein electrophoresis and ... Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA ... In cases where tonsillitis is the precipitating factor for episodic hematuria, a tonsillectomy has been claimed to reduce the ...
Like complement factor H, CFHR5 is able to bind to complement C3. A mutation in CHFR5 was found in patients with the disease ... "Entrez Gene: CFHR5 complement factor H-related 5". McRae JL, Duthy TG, Griggs KM, et al. (2005). "Human factor H-related ... 2006). "Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with ... Complement factor H-related protein 5 is a protein that in humans is encoded by the CFHR5 gene. CFHR5 is structurally related ...
Díaz-Guillén MA, Rodríguez de Córdoba S, Heine-Suñer D (1999). "A radiation hybrid map of complement factor H and factor H- ... Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an expanding family of complement-regulatory proteins ... "Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related ... Complement factor H-related protein 2 is a protein that in humans is encoded by the CFHR2 gene. GRCh38: Ensembl release 89: ...
"Cellular adhesion mediated by factor J, a complement inhibitor. Evidence for nucleolin involvement". The Journal of Biological ... Nucleolin is also able to act as a transcriptional coactivator with Chicken Ovalbumin Upstream Promoter Transcription Factor II ...
These SNPs were located in the gene encoding complement factor H, which was an unexpected finding in the research of ARMD. The ... "Complement factor H variant increases the risk of age-related macular degeneration". Science. 308 (5720): 419-21. doi:10.1126/ ... "Complement factor H polymorphism in age-related macular degeneration". Science. 308 (5720): 385-9. doi:10.1126/science.1109557 ... In addition to the conceptual framework several additional factors enabled the GWA studies. One was the advent of biobanks, ...
In this analysis, it is usually a reduced complement factor C4, rather than the C1-INH deficiency itself, that is detected. The ... especially depletion of complement factors 2 and 4, may indicate deficiency of C1-inhibitor. HAE type III is a diagnosis of ... which encodes the coagulation protein factor XII. All forms of HAE lead to abnormal activation of the complement system, and ... In hereditary angioedema, bradykinin formation is caused by continuous activation of the complement system due to a deficiency ...
Such proteins are referred to as virokines if they resemble cytokines, growth factors, or complement regulators; the term ... The first identified virokine was an epidermal growth factor-like protein found in myxoma viruses. Much of the early work on ... Kotwal, GJ; Moss, B (8 September 1988). "Vaccinia virus encodes a secretory polypeptide structurally related to complement ... which was discovered to secrete proteins that promote proliferation of neighboring cells and block complement immune activity ...
Complement factors are decreased in rheumatoid arthritis and lupus arthritis. Microscopic analysis of synovial fluid is ... Jay, GD; Britt, DE; Cha, CJ (March 2000). "Lubricin is a product of megakaryocyte stimulating factor gene expression by human ...
Able to break down fibrin clots, cleave complement protein C3, and activate Factor XII. ... Faktor XII (Hageman Factor) liver A protein that circulates inactively, until activated by collagen, platelets, or exposed ... The combination and activation of this range of complement proteins forms themembrane attack complex, which is able to insert ... A complex of the complement proteins C5b, C6, C7, C8, and multiple units of C9. ...
Overall this protein shows similarity to the complement 1Q factors (C1Q). However, when the 3-dimensional structure of the ... "The crystal structure of a complement-1q family protein suggests an evolutionary link to tumor necrosis factor". Current ... A low level of adiponectin is an independent risk factor for developing: Metabolic syndrome Diabetes mellitus Lower levels of ... Renaldi O, Pramono B, Sinorita H, Purnomo LB, Asdie RH, Asdie AH (January 2009). "Hypoadiponectinemia: a risk factor for ...
"Evid Based Complement Alternat Med. 7 (1): 11-28. doi:10.1093/ecam/nen023. PMC 2816378. PMID 18955327.. ... Multiple factors such as gender, age, ethnicity, education and social class are also shown to have association with prevalence ... "Who Uses CAM? A Narrative Review of Demographic Characteristics and Health Factors Associated with CAM Use" ...
The genes for the complement system proteins factor H (CFH), factor B (CFB) and factor 3 (C3) are strongly associated with a ... Despriet DD, Klaver CC, Witteman JC, Bergen AA, Kardys I, de Maat MP (2006). "Complement factor H polymorphism, complement ... on chromosome 10 at location 10q26 Complement Factor B/Complement Component 2 (CFB/CC2) on chromosome 6 at 6p21.3 Polymorphisms ... Absence of the complement factor H-related genes R3 and R1 protects against AMD. Two independent studies in 2007 showed a ...
Malhotra R, Ward M, Sim RB, Bird MI (1999). "Identification of human complement Factor H as a ligand for L-selectin". Biochem. ... The molecule is composed of multiple domains: one homologous to lectins, one to epidermal growth factor, and two to the ... The embryo secretes human chorionic gonadotropin (hCG), which downregulates anti-adhesion factor, MUC-1, located on the uterine ...
"Complement Factor H Variant Increases the Risk of Age-Related Macular Degeneration". Science. 308 (5720): 419-21. doi:10.1126/ ... Some of the factors that should be considered are the level of efficacy of various genetic tests in the general population, ... "individual risk factors". Perhaps the most critical issue with the commercialization of personalised medicine is the protection ... there are a number of factors that must be considered. The detailed account of genetic information from the individual will ...
2003) Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the ... 2008) Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on ... 2005) Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal ... a b c Meri, S. (2007) Loss of self-control in the complement system and innate autoreactivity. From Ann N Y Acad Sci 1109, 93- ...
Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... CFHR4 complement factor H-related 4". Hageman GS, Hancox LS, Taiber AJ, et al. (2007). "Extended Haplotypes in the Complement ... Complement factor H-related protein 4 is a protein that in humans is encoded by the CFHR4 gene. GRCh38: Ensembl release 89: ... 2000). "Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and ...
Opsonins include Mfge8, Gas6, Protein S, antibodies and complement factors C1q and C3b. Phagoptosis has multiple functions ... Pathogenic cells such as bacteria can be opsonised by antibodies or complement factors, enabling their phagocytosis and ...
1989). "20 KDa homologous restriction factor of complement resembles T cell activating protein". Biochem. Biophys. Res. Commun ... When complement activation leads to deposition of C5b678 on host cells, CD59 can prevent C9 from polymerizing and forming the ... Ninomiya H, Sims PJ (1992). "The human complement regulatory protein CD59 binds to the alpha-chain of C8 and to the "b"domain ... 1992). "Complement regulatory proteins at the feto-maternal interface during human placental development: distribution of CD59 ...
Lee FJ, Moss J, Vaughan M (1992). "Human and Giardia ADP-ribosylation factors (ARFs) complement ARF function in Saccharomyces ... ADP-ribosylation factor 5 is a protein that in humans is encoded by the ARF5 gene. ADP-ribosylation factor 5 (ARF5) is a member ... Shin, O H; Exton J H (August 2001). "Differential binding of arfaptin 2/POR1 to ADP-ribosylation factors and Rac1". Biochem. ... "Entrez Gene: ARF5 ADP-ribosylation factor 5". Kanoh, H; Williger B T; Exton J H (February 1997). "Arfaptin 1, a putative ...
"Entrez Gene: CFHR3 complement factor H-related 3". Zipfel PF, Skerka C (1994). "Complement factor H and related proteins: an ... Diaz-Guillen MA, Rodriguez de Cordoba S, Heine-Suner D (Jul 1999). "A radiation hybrid map of complement factor H and factor H- ... Complement factor H-related protein 3 is a protein that in humans is encoded by the CFHR3 gene. GRCh38: Ensembl release 89: ... 2000). "Complement factor H: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes ...
This is a type of safeguard to the system, almost like a two-factor authentication method. First, the B cells have to encounter ... Differentiation of mature B cells into plasma cells is dependent upon the transcription factors Blimp-1/PRDM1 and IRF4. ... Immunology: lymphocytic adaptive immune system and complement. Lymphoid. Antigens. *Antigen *Superantigen. *Allergen ...
CFHR5 complement factor H related 5 [Homo sapiens] CFHR5 complement factor H related 5 [Homo sapiens]. Gene ID:81494 ... complement factor H-related protein 5. Names. factor H-related protein 5. ... Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important ... Title: Complement factor H-related hybrid protein deregulates complement in dense deposit disease. ...
Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type ... Structure of a complex between CCPs 6 and 7 of Human Complement Factor H and Neisseria meningitidis FHbp Variant 3 Wild type. * ... Factor H binding protein (fHbp) is a key antigen that elicits protective immunity against the meningococcus and recruits the ... Factor H binding protein (fHbp) is a key antigen that elicits protective immunity against the men ... ...
Complement Factor I Antibody (Internal), Rabbit Anti Human Polyclonal Antibody validated in WB, IHC-P (ALS17749), Abgent ... Complement component factor i, Factor I, FI, KAF, I factor, I factor (complement), C3b-INA, Complement factor I, Light chain of ... Anti-CFI / Complement Factor I Antibody (Internal) is for research use only and not for use in diagnostic or therapeutic ... home , Products , Primary Antibodies , Antibody Collections , GPCR Antibodies , Anti-CFI / Complement Factor I Antibody ( ...
Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation ... Human complement factor H Y402H polymorphism causes an age-related macular degeneration phenotype and lipoprotein dysregulation ... One of the strongest susceptibility genes for age-related macular degeneration (AMD) is complement factor H (CFH); however, its ... but not complement activation, which correlated with the AMD-like phenotype in old CFH-H/H mice. Specifically, apolipoproteins ...
... Jean Charchaflieh,1 Julie Rushbrook,2 Samrat Worah,2 and Ming Zhang ...
Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the ... C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. UniProt ...
Schulz, T. F.; Schwaeble, W.; Stanley, K. K.; Weiss, Elisabeth H.; Dierich, Manfred P. (1986): Human complement factor H. ... kDa fragment of human complement control protein factor H using polyclonal and monoclonal antibodies to screen a human liver ...
Factor H-like 1 (FHL-1) is a splice variant of FH that also possesses complement-inhibiting function. Additional five factor H- ... In addition to its canonical role in complement regulation, several other functions of FH have been discovered and a large ... While other fluid-phase and cell membrane-bound regulators of complement have been identified, FH is essential for controlling ... Moreover, numerous pathogenic microbes and some tumor cells have developed the ability to exploit FH for complement evasion. ...
Buy our Recombinant Human Complement factor B protein (denatured). Ab174401 is a protein fragment produced in Escherichia coli ... Recombinant Human Complement factor B protein (denatured). See all Complement factor B proteins and peptides. ... Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb ... a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in ...
Factor I deficiency in turn leads to low levels of complement component 3 (C3), factor B, factor H and properdin. in plasma, ... complement factor I". Goldberger G, Bruns GA, Rits M, Edge MD, Kwiatkowski DJ (Jul 1987). "Human complement factor I: analysis ... Complement factor I, also known as C3b/C4b inactivator, is a protein that in humans is encoded by the CFI gene. Complement ... Leitão MF, Vilela MM, Rutz R, Grumach AS, Condino-Neto A, Kirschfink M (Dec 1997). "Complement factor I deficiency in a family ...
Complement factor B is a protein that in humans is encoded by the CFB gene. This gene encodes complement factor B, a component ... CFB complement factor B". Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). "The Ba fragment of complement factor B ... Christie DL, Gagnon J (January 1983). "Amino acid sequence of the Bb fragment from complement Factor B. Sequence of the major ... Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the ...
Complement factor H gene associations with end-stage kidney disease in African Americans.. Bonomo JA1, Palmer ND2, Hicks PJ3, ... Mutations in the complement factor H gene (CFH) region associate with renal-limited mesangial proliferative forms of ...
Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H. R D Horstmann, H ... Isolated complement components were used to study the regulation of the alternative complement pathway C3 convertase (EC 3.4. ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ... Antiphagocytic activity of streptococcal M protein: selective binding of complement control protein factor H ...
Staphylococcus aureusClumping Factor A Binds to Complement Regulator Factor I and Increases Factor I Cleavage of C3b ... Myeloperoxidase influences the complement regulatory activity of complement factor H. Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li ... Factor H and Factor H-Related Protein 1 Bind to Human Neutrophils via Complement Receptor 3, Mediate Attachment to Candida ... Acquisition of Complement Inhibitor Serine Protease Factor I and Its Cofactors C4b-Binding Protein and Factor H by Prevotella ...
Browse our Complement Factor H Lysate catalog backed by our Guarantee+. ... Complement Factor H Lysates available through Novus Biologicals. ... Complement Factor H Lysates. We offer Complement Factor H ... factor H lysate, factor H-like 1 lysate, FH lysate, FHL1 lysate, H factor 1 (complement) lysate, H factor 1 lysate, H factor 2 ... Alternate Names for Complement Factor H Lysates. Complement Factor H lysate, CFH lysate, adrenomedullin binding protein lysate ...
Browse our Complement Factor H Antibodies all backed by our Guarantee+. ... Complement Factor H Antibodies available through Novus Biologicals. ... anti-H factor 1 (complement) antibody, anti-H factor 1 antibody, anti-H factor 2 (complement) antibody, anti-HF antibody, anti- ... Complement Factor H Antibodies. We offer Complement Factor H Antibodies for use in common research applications: ELISA, Flow ...
J:277202 Song D, et al., Complement Factor H Mutation W1206R Causes Retinal Thrombosis and Ischemic Retinopathy in Mice. Am J ...
Factor D cleaves factor B when the latter is complexed with factor C3b, activating the C3bbb complex, which then becomes the C3 ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ... "Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D ...
The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ... Complement Factor 9 Deficiency in Serum of Human Neonates J Infect Dis. 1992 Jul;166(1):53-7. doi: 10.1093/infdis/166.1.53. ... The serum concentration of complement factor C9 (C9) was 260 +/- 47 micrograms/ml (+/- SE) in 14 mothers and less than 42 ...
... factor VIII deficiency (ortholog); FOUND IN extracellular space (inferred); secretory granule (inferred) ... INVOLVED IN complement activation, alternative pathway (inferred); ASSOCIATED WITH autistic disorder (ortholog); Experimental ... Homo sapiens (human) : CFP (complement factor properdin) HGNC Alliance Mus musculus (house mouse) : Cfp (complement factor ... Sus scrofa (pig) : CFP (complement factor properdin) Chlorocebus sabaeus (African green monkey) : CFP (complement factor ...
... products and learn more about anti-Complement Factor D/Adipsin, Polyclonal, Novus Biologicals 0.1mg; 0.1mg; Unlabeled. ... Adipsin, ADNcomplement factor D, C3 convertase activator, complement factor D (adipsin), complement factor D preproprotein, D ... component of complement (adipsin), DF, EC 3.4.21, EC 3.4.21.46, PFD, Properdin factor DADIPSIN. ...
Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ... Among 116,204 single-nucleotide polymorphisms genotyped, an intronic and common variant in the complement factor H gene (CFH) ...
Both age and smoking, two important risk factors for AMD, influence plasma levels of complement factor H (28). CFH sequences ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration. By Robert J. Klein, Caroline Zeiss, Emily Y. Chew, Jen- ... Complement Factor H Polymorphism in Age-Related Macular Degeneration Message Subject. (Your Name) has forwarded a page to you ...
CRP-Induced Expression of Complement- Inhibitory Factors Protects HSVECs From Complement-Mediated Injury. To assess whether the ... 13 The effect of CRP on the expression of complement-inhibitory factors is unknown. Because complement-inhibitory proteins ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ... C-Reactive Protein Upregulates Complement-Inhibitory Factors in Endothelial Cells. Shu-Hong Li, Paul E. Szmitko, Richard D. ...
Structures of native and complexed complement factor D: implications of the atypical His57 conformation and self-inhibitory ... R-Factor (R-Work). R-Factor (R-Free). R-Factor (R-Free Error). Percent Reflections (Observed). ... R-Factor (All). R-Factor (Observed). R-Work. R-Free. R-Free Selection Details. ...
  • The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. (nih.gov)
  • Rare Variants in the Complement Factor H-Related Protein 5 Gene Contribute to Genetic Susceptibility to IgA Nephropathy. (nih.gov)
  • Non-functional V1 fHbps were further characterised by binding and structural studies, and shown in non-transgenic and transgenic mice (expressing chimeric fH that binds fHbp and precisely regulates complement system) to retain their immunogenicity. (rcsb.org)
  • Biochemical analyses of aged CFH mice after HFC diet revealed genotype-dependent changes in plasma and eyecup lipoproteins, but not complement activation, which correlated with the AMD-like phenotype in old CFH-H/H mice. (duke.edu)
  • Genetic polymorphism in Factor I has been observed and recently explained in terms of variants R201S, R406H, R502L. (wikipedia.org)
  • The molecular genetics and polymorphism of C2 and factor B". British Medical Bulletin. (wikipedia.org)
  • In the table below is a performance comparison of diseases selected on disease frequency and known heritability estimates, with use of single-nucleotide polymorphism (SNP) based models reflecting known genetic factors for a European population (subject to change as more associations are discovered). (wikipedia.org)
  • The major difference in backbone structure between Factor D and the other serine proteases of the chymotrpsin family is in the surface loops connecting the secondary structural elements. (wikipedia.org)
  • CD46) and factor H with the development of atypical hemolytic uremic syndrome (aHUS) ( 9 , 10 ). (jimmunol.org)
  • In summary, this study shows that enhancive miR-146a can upregulate the inflammatory factor IL-1β in chronic TLE by downregulating CFH, and that upregulation of IL-1β plays an important feedback-regulating role in the expression of miR-146a and CFH, forming a miR-146a-CFH-IL-1β loop circuit that initiates a cascade of inflammation and then leads to the perpetuate inflammation in TLE. (biologists.org)
  • if C3b is bound to a bacterium, factor B will bind and the cascade will be set off as normal. (wikipedia.org)