Complement and related clinical disorders

The complement system, composed of 20 plasma proteins and several membrane receptors, plays an essential role in humoral immune responses. The activation of the classical and/or alternative pathways by specific and non-specific stimuli leads to the generation of chemotactic and anaphylatoxic inflammatory mediators, the formation of the cytolytic membrane attack complex, and the formation of C3 breakdown fragments which are opsonic. There is now evidence that the membrane receptors play important immunoregulatory functions. The link between various disease states and deficiencies of complement components and membrane receptors further supports the important immunological role this system plays. This review briefly introduces the components of the system, their biological roles and diseases associated with deficiency states.