作者: Péter Gál , Géza Ambrus , Zsolt Lőrincz , Péter Závodszky
关键词: Cell biology 、 Complement system 、 Serine protease 、 Lectin pathway 、 Classical complement pathway 、 Mannan-binding lectin 、 Proteases 、 Chemistry 、 C1-inhibitor 、 Serpin
摘要: Various antigen structures initiate the classical and lectin pathways of complement activation. Multidomain modular proteases (C1r, C1s MASPs) are involved in initiation complexes both pathways. Despite identical domain organization these serine there differences their specificities functions. A comparative analysis is given on similarities mechanism action proteases, with emphasize structural aspects. Recent functional data underline significance molecular dynamics activation process C1 MBL/MASPs. While role MASP-2 pathway supported by increasing number evidence, same can not be said about MASP-1 MASP-3. The pathological processes such as inflammation subsequent repair processes, apoptosis, susceptibility to infectious diseases etc. also reviewed. inhibitor essential regulating This serpin interacts several including C1r, MASPs. deficiency related diseases, a therapeutic agent discussed.