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Terminal Complement Pathway Deficiency
Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC). It involves deficiencies of C5, C6, C7, and C8. (While C9 is part of the MAC, and deficiencies have been identified, it is not required for cell lysis.) People with this condition are prone to meningococcal ''Neisseria meningitidis'', often referred to as meningococcus, is a Gram-negative bacterium that can cause meningitis and other forms of meningococcal disease such as meningococcemia, a life-threatening sepsis. The bacterium is referred to as a ... infection. Vaccination may be recommended. Cause Diagnosis Suspect terminal complement pathway deficiency with patients who have more than one episode of Neisseria infection. Initial complement tests often include C3 and C4, but not C5 through C9. Instead, the CH50 result may play a role in diagnosis: if the CH50 level is low but C3 and C4 are normal, then analysis of the individual terminal components ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Complement Membrane Attack Complex
The membrane attack complex (MAC) or terminal complement complex (TCC) is a complex of proteins typically formed on the surface of pathogen cell membranes as a result of the activation of the host's complement system, and as such is an effector of the immune system. Antibody-mediated complement activation leads to MAC deposition on the surface of infected cells. Assembly of the MAC leads to pores that disrupt the cell membrane of target cells, leading to cell lysis and death. The MAC is composed of the complement components C5b, C6, C7, C8 and several C9 molecules. A number of proteins participate in the assembly of the MAC. Freshly activated C5b binds to C6 to form a C5b-6 complex, then to C7 forming the C5b-6-7 complex. The C5b-6-7 complex binds to C8, which is composed of three chains (alpha, beta, and gamma), thus forming the C5b-6-7-8 complex. C5b-6-7-8 subsequently binds to C9 and acts as a catalyst in the polymerization of C9. Structure and function MAC is composed ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Complement Component 5
Complement component 5 is a protein that in humans is encoded by the ''C5'' gene. Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b: * C5a plays an important role in chemotaxis. * C5b forms the first part of the complement membrane attack complex. Deficiency is thought to cause Leiner's disease. Function Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. This protein is composed of alpha and beta polypeptide chains that are linked by a disulfide bridge. An activation peptide, C5a, which is an anaphylatoxin that possesses potent spasmogenic and chemotactic activity, is derived from the alpha polypeptide via cleavage with a C5-convertase. The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components. ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Complement Component 6
Complement component 6 is a protein that in humans is encoded by the ''C6'' gene. Complement component 6 is a protein involved in the complement system The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and at .... It is part of the membrane attack complex which can insert into the cell membrane and cause the cell to lyse. People with C6 deficiency are prone to bacterial infection. References Further reading * * * * * * * * * * * * * * * * * * * External links * Complement system {{gene-5-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Complement Component 7
Complement component 7 is a protein involved in the complement system of the innate immune system. C7 is part of the membrane attack complex (MAC) which creates a hole on pathogen surfaces, leading to cell lysis and death. Its primary task is to bind the C5bC6 complex together. This junction alters the configuration of the protein molecules, exposing a hydrophobic site on C7 that allows the C7 to insert into the phospholipid bilayer The lipid bilayer (or phospholipid bilayer) is a thin polar membrane made of two layers of lipid molecules. These membranes are flat sheets that form a continuous barrier around all cells. The cell membranes of almost all organisms and many vir ... of the pathogen. See also * Terminal complement pathway deficiency External links * * References Complement system {{gene-5-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
C8 Complex
Complement component 8 is a protein involved in the complement system. It is part of the membrane attack complex (MAC). A hereditary deficiency of C8 can result in increased susceptibility to Neisseria infections, such as meningitis and gonorrhea Gonorrhea, colloquially known as the clap, is a sexually transmitted infection (STI) caused by the bacterium ''Neisseria gonorrhoeae''. Infection may involve the genitals, mouth, or rectum. Infected men may experience pain or burning with u .... Structure C8 is a heterotrimer; it consists of three different subunits. These are called C8 alpha, beta and gamma chains, encoded by the genes ''C8A'', ''C8B'' and ''C8G'' respectively. References External links * Complement system {{gene-9-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Complement Component 9
Complement component 9 (C9) is a MACPF protein involved in the complement system, which is part of the innate immune system. Once activated, about 12-18 molecules of C9 polymerize to form pores in target cell membranes, causing lysis and cell death. C9 is one member of the complement membrane attack complex (MAC), which also includes complement components C5b, C6, C7 and C8. The formation of the MAC occurs through three distinct pathways: the classical, alternative, and lectin pathways. Pore formation by C9 is an important way that bacterial cells are killed during an infection, and the target cell is often covered in multiple MACs. The clinical impact of a deficiency in C9 is an infection with the gram-negative bacterium ''Neisseria meningitidis.'' Structure C9 genes include 11 exons and 10 introns An intron is any nucleotide sequence within a gene that is not expressed or operative in the final RNA product. The word ''intron'' is derived from the term ''intragenic ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Meningococcal
''Neisseria meningitidis'', often referred to as meningococcus, is a Gram-negative bacterium that can cause meningitis and other forms of meningococcal disease such as meningococcemia, a life-threatening sepsis. The bacterium is referred to as a coccus because it is round, and more specifically a diplococcus because of its tendency to form pairs. About 10% of adults are carriers of the bacteria in their nasopharynx. As an exclusively human pathogen, it is the main cause of bacterial meningitis in children and young adults, causing developmental impairment and death in about 10% of cases. It causes the only form of bacterial meningitis known to occur epidemically, mainly in Africa and Asia. It occurs worldwide in both epidemic and endemic form. ''N. meningitidis'' is spread through saliva and respiratory secretions during coughing, sneezing, kissing, chewing on toys and through sharing a source of fresh water. It has also been reported to be transmitted through oral sex and cause ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Total Complement Activity
Total complement activity (TCA) refers to a series of tests that determine the functioning of the complement system in an individual. Tests A variety of tests can be used to measure TCA, but the most commonly used on is the CH50 test. Other tests include the liposome immunoassay (LIA), single tube titration method, and the plate-hemolysis method. CH50 Procedure The test is based on the capacity of an individual's serum to lyse sheep erythrocytes coated with anti-sheep antibodies (preferably rabbit IgG). The individual's serum is diluted until the minimum concentration is reached that 50% of the sheep red blood cells are lysed, and that concentration is recorded as the CH50. The CH50 is testing the classical complement pathway in an individual thus requiring functioning C1-C9 factors. CH50 Interpretation If an individual has deficient or malfunctioning complement factors, then at a baseline they have decreased capacity to lyse the erythrocytes. Therefore, any dilution t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
