医学免疫学英文版课件：Chapter 6 Complement system.pptxVIP

MEDICAL IMMUNOLOGYChapter 6 Complement System ContentsIntroduction of the complement systemActivation pathways of complement system ※Regulation of complement activationBiological functions of complement ※ Jules Bordet (1870-1961), 1919 Noble Prize Definition of complement system：A system of serum and cell surface proteins （including more than 30 proteins ) that interact with one another and with other molecules of the immune system to generate important effectors of innate and adaptive immune response. PartⅠ Components and properties of complement system(I) The components of complement systemComponents participating complement activationClassical pathway: C1 (C1q,C1r,C1s)，C2， C3， C4 MBL（mannose-binding lectin) pathway: MBL, MASP( MBL-associated serine protease)Alternative pathway: factor B, factor DCommon terminal pathway：C5，C6，C7，C8，C9 2. Regulatory componentsfactor I, factor H, properdin (factor P)S protein, SP40/40C1 inhibitor (C1 INH)C4-binding protein (C4BP)membrane cofactor protein (MCP)decay accelerating factor (DAF)homologous restriction factor (HRF)membrane inhibitor of reactive lysis (MIRL 3.Complement receptors (CR) CR1~CR5, C3aR, C2aR, C4aR (Ⅱ) Physical and chemical features complement systemSynthesized sites: liver, macrophage, small intestine epitheliumThe concentration of complement in serum is stable (4 g/L) , C3 is the highest in all of complement components:1~2 g/LHeat–labile feature: 56℃ 30min—inactivation 0~10℃ for 3~5 days (Ⅲ) Nomenclature of the complementsystemIntrinsic components in classical pathway: C1~C9Intrinsic components in alternative pathway: facto factor DRegulatory proteins: C1 INH, C4BPCleaved fragments: C3a, C3b; C2a, C2bActivated components: C1; C4b2aInactivated components: iC3bComplement receptor: CR PartⅡ Activation of the complement system (Ⅰ) The Classical pathwayComponents: C1(C1q,C1r,C1s),C4,C2,C3,5,6,7,8,9Activating substances: Ag-Ab complex or immune complexIgG1-3, IgM 3. Process of the classical pathwayInitiation ste