免疫05-Complement.ppt

Activation of the Complement System Terminal pathway: MAC(membrane attack complex) Activation of the Complement System Terminal pathway a a Regulation of complement activation 1.Self-regulation C4b2a, C3bBb, C4b, C3b, C5b 2.Control proteins Classical pathway:C1INH, C4bp, I, MCP, DAF Alternative pathway: H, I, CR1, DAF, P Formation of MAC: C8bp, CD59 Regulation of complement activation C1IHN缺陷引起血管神经性水肿 C4bp（C4 Binding Protein) Regulation of complement activation Regulation of complement activation Regulation of complement activation 膜结合型的补体调节蛋白 Regulation of complement activation Biologic function of complement system 1.complement mediates anti-infection immunity (1) Lysis of cell or microorganisms (2) opsonization (3) inflammation (1)complement mediated the lysis of cells (2) Opsonization opsonin: C3b, C4b, iC3b cell: phagocyte receptor: CR1, CR3, CR4 function: enhance phagocytosis (3) inflammatory response symptoms: redness, swelling, heat and pain inflammation mediators: C5a, C3a, C4a ( anaphylatoxins) C5a (chemotaxis) Biologic function of complement system 2. Complement maintains homeostasis clearance of IC: C3b elimination of apoptosis cells C1q, C3b, iC3b 3. Complement mediates adaptive immunity (1) induces the immune response (2) mediates the proliferation and differentiation of immune cells (3) mediates the effect of immune cells (4) mediates the immune memory 4. Complement interacts with other enzyme systems 补体缺陷的临床表现 临床表现 主要相关的缺陷补体成分 次要相关的缺陷补体成分 遗传性血管神经性水肿 C1INH 严重顽固性皮肤损害 C1q 反复发作性细菌感染 C3，I因子 C1r，C1q 免疫复合物性血管炎（包括肾炎） C1q，C1r，C4，C2 C3，C5 反复发作性革兰氏阳性球菌感染 C5，C6，C7，C8 系统性红斑狼疮 CR1 C1q分子的C端球形结构是与Ig上的补体 结合位点相结合的部位，它的启动可使 C1r构型改变，成为具有活性的C1r并诱 导C1s的活化，成为具有酯酶活性的