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Complement, initially described as a single substance necessary for antibody dependent bacteriolysis, has emerged as the major humoral effector system of host defense. The system consists of more than 30 proteins (Table 1) that exhibit an impressive conservation of a limited number of structural motifs or domains. This conservation indicates that multiple exon shuffling and gene duplication events occurred during evolution (1). Fifteen plasma proteins participate in the activation of complement, serving as enzymes, enzyme cofactors, or precursors of biologically active fragments. When activated, these proteins mediate acute 277 VOLANAKIS Table 1 Proteins of the complement systema Functional Group Prevalent form Participating in in native state Serum soluble activation sequences C l q, C lr, C is, D, MBP,MASP, C4, C3, C2, B, C5, C6, C7, C8, C9 Regulatory C l INH, C4BP, H. I. P C3a!C5a INA Receptors S protein CRI, DAF. MCP HRF.CD59 ClqR. C5aR CRl, CR2. CR3 Membrane associated aEstablished symbols have been used for most complement proteins. In addition, the followi ng generally accepted abbreviations have been used: INH, inhibitor; C4BP. C4b-binding protein; INA. MASP, inactivator; R, receptor, e.g., CRI, complement receptor type I; membrane cofactor protein; HRF, homologous restriction factor. mannose-binding protein associated
Annual Review of Immunology – Annual Reviews
Published: Apr 1, 1995
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