Anti-C1 inhibitor antibody [HL1196] - BSA and Azide free

Supplementary info

Activity summary

C1 inhibitor also known as C1 esterase inhibitor or C1-INH is a serpin family protein that plays a significant role in inhibiting the complement system. This protein found in human serum has a molecular mass of about 104 kDa. C1 inhibitor is predominantly expressed in the liver and secreted into the bloodstream where it functions to regulate several proteolytic cascades. As a glycoprotein it also contains significant carbohydrate moieties that aid in its stability and function.

Biological function summary

As an inhibitor of the classical pathway of the complement system C1 inhibitor controls the activation of C1 esterase. This interaction prevents the uncontrolled activation of downstream components of the complement cascade which could result in tissue damage. C1 inhibitor also participates in regulating the kallikrein-kinin system preventing excessive inflammation. It achieves these roles not alone but as a part of complexes that interact with other proteins to maintain homeostasis within the immune system.

Pathways

The role of C1 inhibitor intersects with critical inflammatory modulatory pathways such as the complement pathway and the kallikrein-kinin system. These pathways are essential for proper immune response modulation preventing excessive inflammation and maintaining vascular permeability. C1-INH acts alongside proteases like kallikrein and proteins like C4 and C2 within these pathways to regulate the initiation and progression of the immune response during infections or insults.

Associated diseases and disorders

Inadequate levels or functional defects in C1 inhibitor lead to hereditary angioedema a condition characterized by recurrent episodes of severe swelling. This disease highlights the importance of C1-INH in controlling vascular leakage and inflammation. Moreover C1 inhibitor has been implicated in autoimmune diseases like systemic lupus erythematosus where its regulatory kinetics with proteins such as C1q play a part in managing excessive complement activation. These associations highlight the important protective role of C1 inhibitor in health and disease.