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Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. [4] Because of redundancies in the immune system , many complement disorders are never diagnosed.
The cleavage of the C4 results in C4b bearing a thioester functional group [-S-C(O)-]: work in the 1980s on C3, and then on C4, indicated the presence, within the parent C3 and C4 structures, of a unique protein modification, a 15-atom (15-membered) thionolactone ring serving to connect the thiol side chain of the amino acid cysteine (Cys) in a ...
Complement 4 deficiency; Specialty: Immunology: Complement tests C4 ... Complement 4 deficiency is a genetic condition affecting complement component 4. [1]
Complement component 5 is a protein that in humans is encoded by the C5 gene. [5] Complement component 5 is involved in the complement system. It is cleaved into C5a and C5b: C5a plays an important role in chemotaxis. [6] C5b forms the first part of the complement membrane attack complex. Deficiency is thought to cause Leiner's disease.
Deficiency in the C1q protein of the classical complement pathway can lead to development of systemic lupus erythematosus. [2] [16] Among the many functions of C1q, C1q triggers clearance of immune complexes and apoptotic cells by activating the classical pathway and binding directly onto phagocytes.
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, [1] it is not required for cell lysis. [2]) People with this condition are prone to meningococcal infection. [3]
B cells low but present or normal, but with reduction in 2 or more isotypes (usually IgG & IgA, sometimes IgM): common variable immunodeficiency (CVID), CD19 deficiency, TACI (TNFRSF13B) deficiency, BAFF receptor deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgM syndromes
Complement component 4B (Chido blood group) is a kind of the Complement component 4 protein that in humans is encoded by the C4B gene. [ 5 ] This gene encodes the basic form of complement factor 4, part of the classical activation pathway.