Primary deficiencies of the complement system Radana Zachova Institute of Immunology Faculty hospital Prague,Motol
Primary deficiencies of the complement system Radana Zachová Institute of Immunology Faculty hospital Prague, Motol
Complement system part of hummoral innate immune system group of serum and cell surface proteins important in antiinfectious and inflammatory immune response activated by a cascade of reactions during activation generates active components
Complement system • part of hummoral innate immune system • group of serum and cell surface proteins • important in antiinfectious and inflammatory immune response • activated by a cascade of reactions • during activation generates active components
Complement activation pathways Alternative pathway (pathogen surface) Mannan binding lectin pathway (pathogen surface) Classical pathway_(antigen-antibody complexes)
Complement activation pathways • Alternative pathway (pathogen surface) • Mannan binding lectin pathway (pathogen surface) • Classical pathway (antigen-antibody complexes)
Complement activation pathways Antigen-antibody complexes Microbial surfaces Microbial surfaces Classical pathway Mannanbinding lectir Alternativepathway pathway C1q MBL c3 C1r-C1s MASP1-MASP2 B C4 C4 0 c2 C2 C3 Opsonic fragments, Vasoactive and chemotactic C3b,ic3b cs fragments,C4a,C3a,C5a C6 C7 Immune response stimulating fragments, C3d 9 Membrane-attack complex (MAC)
Complement activation pathways
Primary complement deficiencies 1)Components of activation pathways C1q,C1r,C1s,C4,C2,C3,MBL,D,B,C5,C6,C7,C8,C9 2)Control proteins soluble control proteins C1 inhibitor,factor I,factor H,C4b binding protein,S protein, SP-40,40 membrane regulatory proteins CD 55(DAF),MCP CD 46,CD 59,HRF/C8bp 3)Receptors for complement C1q receptor,CR1(CD 35),CR2(CD21),CR3(CD11b/CD18),CR4 (CD11c-CD18)
Primary complement deficiencies 1) Components of activation pathways C1q, C1r,C1s,C4,C2,C3, MBL, D,B,C5,C6,C7,C8,C9 2) Control proteins • soluble control proteins C1 inhibitor, factor I, factor H,C4b binding protein,S protein, SP-40,40 • membrane regulatory proteins CD 55(DAF), MCP CD 46, CD 59, HRF/C8bp 3) Receptors for complement C1q receptor, CR1(CD 35), CR2 (CD21), CR3 (CD11b/CD18), CR4 (CD11c-CD18)