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A protein complex composed of 18 complement component 1q (C1q) subcomponent chains: six C1qA, six C1qB and six C1qC. The C1q complex is part of the complement component C1 complex (C1 complex) and undergoes confirmational rearrangement following binding of pentraxins or antigen-antibody complexes that include either immunoglobulin M (IgM) or several IgG molecules. These confirmational changes activate the C1r chains of the C1 complex, which, in turn, cleaves C1s chains of the C1 complex. Following C1s cleavage, the C1 complex can proteolytically activate C2 and C4 and promotes formation of C3-convertase, which cleaves C3 and activates the classical complement pathway.
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Complement component-4 (1744 aa, ~193 kDa) is part of the complement cascade, which mediates the innate immune response to infection. The full length protein is a precursor that is secreted as a trimeric molecule containing an alpha, beta and gamma chain. When the complement cascade is initiated, the protein is cleaved. This proteolysis releases both the alpha chain, C4 anaphylatoxin, which stimulates inflammation, and a dimer of the beta and gamma chains, which mediates the interaction between the antigen-antibody complex and other complement components.
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A derivative of the natural macrocyclic lactone sirolimus with immunosuppressant and anti-angiogenic properties. In cells, everolimus binds to the immunophilin FK Binding Protein-12 (FKBP-12) to generate an immunosuppressive complex that binds to and inhibits the activation of the mammalian Target of Rapamycin (mTOR), a key regulatory kinase. Inhibition of mTOR activation results in the inhibition of T lymphocyte activation and proliferation associated with antigen and cytokine (IL-2, IL-4, and IL-15) stimulation and the inhibition of antibody production. (NCI05)
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Galectin-3 (250 aa, ~26 kDa) is encoded by the human LGALS3 gene. This protein plays a role in both antibody-mediated immunity and carbohydrate binding.
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IgA is the immunoglobulin subclass that is associated with antibody-mediated mucosal immunity. It is secreted as dimers into the mucosa and is the most effective isotype at fixing complement by the alternative pathway, even though it lacks the ability to fix complement by the classical pathway. There are two subtypes in the human - IgA1 and IgA2.
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The most abundant immunoglobulin G (IgG) subclass in the circulation of healthy individuals. The IgG1 core hinge sequence contains 15 amino acids and two interchain disulfide bonds. This subtype has high affinity for binding both Fc receptors and complement component C1q. IgG1 is the predominant antibody subclass that responds to antigens derived from both soluble and membrane proteins. Reduced levels of circulating IgG1 can indicate the presence of a variety of primary and secondary antibody deficiencies, which can result in hypogammaglobulinemia.
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Immunoglobulin Light Chain Kappa is one of two Ig light chain types. Each Ig molecule is typically composed of two heavy chains and two light chains. Ig kappa light chains contain a variable and a constant region. Kappa light chains are encoded by multiple V, J, and C gene segments, which are separated in the genomes of cells undifferentiated with regard to antibody gene expression. Diversity in the kappa light chain V region arises during differentiation of an activated (kappa-type) antibody-producing B-cell from variable recombination of kappa V genes with kappa J genes and somatic point mutation, which plays a major role in antibody diversity. (from OMIM and NCI)
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Immunoglobulin Light Chain Lambda is one of two Ig light chain types. Each Ig molecule is typically composed of two heavy chains and two light chains but Ig lambda light chains contain a variable and a constant region. Lambda light chains are encoded by multiple V, J, and C gene segments, which are separated in the genomes of cells undifferentiated with regard to antibody gene expression. During differentiation of an activated (lambda-type) antibody-producing B-cell, diversity in the lambda light chain V region arises from variable recombination of lambda V genes with lambda J genes and somatic point mutation, which plays a major role in antibody diversity. (from OMIM 147220 and NCI)
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A natural macrocyclic lactone produced by the bacterium Streptomyces hygroscopicus, with immunosuppressant properties. In cells, sirolimus binds to the immunophilin FK Binding Protein-12 (FKBP-12) to generate an immunosuppressive complex that binds to and inhibits the activation of the mammalian Target Of Rapamycin (mTOR), a key regulatory kinase. This results in inhibition of T lymphocyte activation and proliferation that occurs in response to antigenic and cytokine (IL-2, IL-4, and IL-15) stimulation and inhibition of antibody production. (NCI04)
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Qualitative or quantitative marker of active hepatitis C infection.
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Along with the immunoglobulin G4 subclass (IgG4), the IgG3 subtype makes up a minor part of circulating antibodies in healthy individuals. The core hinge sequence contains approximately four times the number of amino acids as the other subclasses; allotypes of IgG3 can have up to 62 hinge residues and up to four interchain disulfide bonds. The increased hinge size for IgG3 increases the separation between the Fc (constant fragment) tail and Fab (antigen binding fragment) arms of antibodies of this subclass. This enhances the relative flexibility of the molecule, which may modulate antigen-binding capacity and immune complex formation, and may increase the affinity for binding to both Fc receptors and complement component C1q.
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Along with the immunoglobulin G3 subclass (IgG3), the IgG4 subtype makes up a minor part of circulating antibodies in healthy individuals. The IgG4 core hinge sequence contains 12 amino acids and two interchain disulfide bonds.This subtype has moderate affinity for binding Fc receptors and does not bind complement component C1q. The production of IgG4 can be stimulated following repeated or long-term exposure to antigen. Elevated levels are found in various diseases and infections and can be indicative of an fibroinflammatory syndrome known as IgG4-related disease.
