Author: Erik J.M. Toonen

Publisher: Frontiers Media SA

Published: 2023-12-14

Total Pages: 364

ISBN-13: 2832540880

The complement system is an important innate immune surveillance network that has a key role in protecting our bodies against pathogens and other threats. It is a highly complex system consisting of approximately 50 soluble and cell surface-bound proteins that interact to eliminate danger signals. These signals include factors such as invading microorganisms, necrotic cells, and immune complexes. Furthermore, complement can link innate and adaptive immune responses by regulating T cell and B cell responses. The complement system is tightly regulated to avoid uncontrolled activation. Dysregulation of the complement system has been linked to numerous diseases, both rare and common. Uncontrolled or exaggerated activation can lead to life-threatening conditions such as dysregulation of coagulation, fibrinolysis, systemic inflammation and shock, and possible failure of a variety of organs such as the eyes, kidneys, skin, brain, and vascular system. In recent years, the complement system has gained increased interest, especially as a promising candidate for therapeutic intervention. Eculizumab, the first approved complement inhibitor, is highly effective for treating atypical hemolytic uremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). Many more drug candidates are currently evaluated in ongoing clinical trials.