Biosynthesis of complement components

Abstract

Many of the serum complement components are synthesized primarily in the liver but extrahepatic synthesis in mononuclear phagocytes has been noted. This local production of complement, particularly at sites of inflammation, may be of importance in host defenses and immunopathological reactions. Recent studies of complement biosynthesis in vitro have revealed some of the genetic and microenvironmental factors that affect the rates of complement synthesis, posttranslational modification, and secretion of these proteins. The third, fourth, and fifth complement components are synthesized as single chain, procomplement proteins that require proteolytic cleavage to generate the native C4 protein is mediated by a plasmin-like enzyme. These studies, plus the most recent developments in analysis of the complement genes at a molecular level, will provide more complete understanding the mechanisms of regulation of complement production.