Complement system in diseases

The complement system is a network of proteins that acts as one of the body's first lines of defense, rapidly tagging pathogens and damaged cells for destruction before the adaptive immune response can mobilize. When its regulation breaks down, however, the same cascade that protects healthy tissue can turn against it, driving conditions such as atypical hemolytic uremic syndrome and other thrombotic microangiopathies, where uncontrolled complement activation damages small blood vessels and triggers clotting. Researchers are working to map the genetic variants and regulatory failures that tip the system toward disease, and therapies like eculizumab — a monoclonal antibody that blocks a central complement protein — have validated this pathway as a tractable drug target while also revealing how much remains unknown about why some patients respond and others do not. Open questions include how complement cross-talks with coagulation factors such as ADAMTS13, and how to design interventions precise enough to restrain pathological activation without compromising the immune protection the system was built to provide.

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Keywords

ComplementImmuneThrombotic MicroangiopathiesHemolytic Uremic SyndromeEculizumabInnate Immunity