complement deficiency

(redirected from C1r)

complement deficiency

A state in which any of the complement proteins is subnormal
Complement deficiencies–associated disorders
C1r    SLE, renal disease, repeated infections
C1s    SLE
C2    SLE, vasculitis, membranoproliferative glomerulonephritis, dermatomyositis
C3 Repeated infections
C5 SLE, gonococcal disease
C6 Relapsing meningococcal meningitis, gonococcal infection
C7 Raynaud's disease, chronic renal disease, gonococcal infection
C8 SLE, gonococcal infection


a complex series of enzymatic proteins occurring in normal serum that are triggered in a cascade manner by, and combine with, the antibody-antigen complexes, producing lysis when the antigen is an intact cell. Complement comprises 25 to 30 discrete proteins, labeled numerically as C1 to C9, and by letters, i.e. B, D, P, etc., and with C1 being divided into subcomponents C1q, C1r and C1s. Components C3 and C5 are involved in the generation of anaphylatoxin and in the promotion of leukocyte chemotaxis, the result of these two activities being the inflammatory response. C1 and C4 are involved in the neutralization of viruses. The components also combine in various sequences to participate in other biological activities, including antibody-mediated immune lysis, phagocytosis, opsonization and anaphylaxis. The complement system is known to be activated by the immunoglobulins IgM and IgG.

alternate complement pathway, alternative complement pathway
the sequence in which complement components C3 and C5 to C9 are activated without participation by C1, C2 and C4 or the presence of an antibody-antigen complex.
complement cascade
the sequence of reactions, each being the catalyst for the next, that leads to the terminal complement pathway and cell lysis. There are two pathways for activation of C3, the 'classical' (below) and the 'alternate' (above).
classical complement pathway
the one in which all of the complement components C1 to C9 participate and is triggered by antibody-antigen complexes.
complement deficiency
various complement components may be deficient without serious effects on the host. C3 deficiency is most severe and occurs in humans, Brittany spaniels and Finnish-Landrace lambs. Increased susceptibility to infections results.
complement fixation tests
utilize antibody-antigen reaction and result in hemolysis to determine the presence of various organisms in the blood. Involves two stages. In the first, also referred to as the test system, antigen is mixed usually with serial dilutions of a test serum in the presence of complement. If the serum contains antibody, i.e. is positive, an antibody-antigen complex is formed which also binds (fixes) complement. In the second stage, also called the indicator system, sheep red blood cells coated with specific, usually rabbit anti-sheep red blood cell antibody are added. The red blood cells are said to be sensitized. If antibody was not present in stage 1, then the free complement lyses the sensitized sheep red blood cells. The basis of many serological tests including those for glanders, tuberculosis and contagious bovine pleuropneumonia. Called also Bordet-Gengou phenomenon. See also immunity.
complement regulatory proteins
a set of at least seven proteins that are present in plasma (C1 INH, C4b-binding protein, factor H and factor I) or present in cell membranes (decay-accelerating factor [DAF], membrane cofactor protein [MCP] and homologous restriction factor [HHF]) that modulate the complement proteins and protect 'innocent' bystander cells and tissues from complement damage.
terminal complement pathway
the final stages of complement activation in which C5, C6, C7, C8 and C9 are activated; common to both the alternate and classical pathways.
References in periodicals archive ?
2 Coagulation factor XI (c) Complement C1r (c) Actin (c) Complement C3 (c) Complement C4B (c0 Talin-1 (c) Apolipoprotein B-100 (c) Complement C5 (c) Ceruloplasmin (c) Alpha-1 antichymotrypsin (c) Inter alpha trypsin inhibitor (c) Kallistratin (c) Corticosteroid-binding globulin (c) Serum paraoxonase (c) Fibronectin (c) Plasmonogen like protein A (c) Complement C2 (c) Fibrinogen alpha chain (c) Extracellular matrix protein-1 (c) Collagen alpha 3 (c) Protein disulphide isomerase A3 (c) LFQ LC-MS/MS Fold change (a) T = 24 h vs Protein description T = 30 min Fibrinogen gamma chain 1.
For a selection of other shows that use "then" see Three Lords and Ladies of London, G1v; Battle of Alcazar, A2v; Locrine, A3r, C2r, E3r, Glv; Warning for Fair Women, D1r, E3v, G3r; Whore of Babylon, A3v, C1r, G2v; White Devil, D4v; Four Plays in One, 8D4v, 8E2v, 8F3r; Duchess of Malfi, H1v; Herod and Antipater, C3r, F3v, F4r, I4v; Fatal Contract, E3v; Lovesick Court, I2v.
C1r, and Henry Wilkinson, The Debt Book (London 1625), esp.
Forming cups with C1R instead of PEI also resulted in a 10 percent increase in cup weight.
Complement system contains several serine proteases such as C1r, C1s, C3 convertase and C5 convertase.
El inhibidor del C1 es una serina proteasa con una amplia variedad de funciones, actua como inhibidor en la via clasica del complemento de la proteasa C1r, C1s y la lectina proteasa ligadora de manosa 1 y 2, en el sistema de contacto bloquea la calicreina plasmatica, el factor XII y el XIa, ademas actua en el sistema fibrinolitico inactivando la plasmina (10, 11).
b2r, b4r, c1r, c5v, d6v, e8v, f8v, g4r, h6v, q3r), while by contrast, some break up syntactical units (such as "kyng Arthur" in HM 136, fol.
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ii) c1R = clA where A is r-open such that R [subset] A [subset] cl(R).
The cotton fabrics (samples C1R and C1F) have rather low resistance to water vapour transfer due to the absence of elastane component and lower structural densities.
Biadego largely based his attribution on the `b' in two Poliphilo woodcuts (folios a6v, c1r, fig.
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