Complement system in diseases

The complement system is a network of proteins that acts as one of the body's first lines of defense, recognizing pathogens and damaged cells and triggering a rapid inflammatory response without requiring prior immune exposure. When its regulation breaks down, complement activation can turn against the body's own tissues, driving conditions such as atypical hemolytic uremic syndrome and other thrombotic microangiopathies, where uncontrolled complement activity damages small blood vessels and leads to organ failure. The development of targeted inhibitors like eculizumab, which blocks a key amplification step in the cascade, has transformed treatment for some of these disorders and confirmed that complement dysregulation is causally central rather than incidental. Researchers are now working to understand why regulatory failures occur in specific vascular beds, how complement interacts with coagulation pathways involving proteins like ADAMTS13, and whether more precisely targeted therapies can achieve disease control with fewer systemic side effects.

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Keywords

ComplementImmuneThrombotic MicroangiopathiesHemolytic Uremic SyndromeEculizumabInnate Immunity