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Complement Regulation
Author(s) -
Meri S.,
Jarva H.
Publication year - 1998
Publication title -
vox sanguinis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.68
H-Index - 83
eISSN - 1423-0410
pISSN - 0042-9007
DOI - 10.1111/j.1423-0410.1998.tb05434.x
Subject(s) - complement system , paroxysmal nocturnal hemoglobinuria , complement (music) , hereditary angioedema , alternative complement pathway , immunology , clearance , complement receptor , complement deficiency , classical complement pathway , cytolysis , complement component 5 , eculizumab , decay accelerating factor , medicine , biology , antibody , genetics , phenotype , cytotoxic t cell , complementation , in vitro , urology , gene
Because of its strong potential for generating inflammation and causing tissue destruction the complement system has to be kept strictly under control. Cells of the host need special protection against the cytolytic complement system. This paper will describe how inappropriate activation of complement in the fluid phase is prevented and how viable human blood cells defend themselves against being destroyed and cleared away by the complement system. Since disturbances in complement regulation occasionally result in disease a brief reference will be made to two of the syndromes caused by complement regulator deficiency, hereditary angioedema (HAE) and paroxysmal nocturnal hemoglobinuria (PNH).