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Transcriptional Regulation of Complement Genes

Transcriptional Regulation of Complement Genes 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Annual Review of Immunology Annual Reviews

Transcriptional Regulation of Complement Genes

Annual Review of Immunology , Volume 13 (1) – Apr 1, 1995

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Publisher
Annual Reviews
Copyright
Copyright 1995 Annual Reviews. All rights reserved
Subject
Review Articles
ISSN
0732-0582
eISSN
1545-3278
DOI
10.1146/annurev.iy.13.040195.001425
pmid
7612224
Publisher site
See Article on Publisher Site

Abstract

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

Journal

Annual Review of ImmunologyAnnual Reviews

Published: Apr 1, 1995

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