SERPING1 Human HEK

SERPING1 is a highly glycosylated plasma protein that plays a significant role in controlling the activation of the complement system. The complement system is a part of the immune system that enhances the ability of antibodies and phagocytic cells to clear pathogens from an organism. SERPING1 specifically inhibits the activated forms of C1r and C1s proteases, which are components of the first complement component, C1 .

The protein forms a proteolytically inactive stoichiometric complex with these proteases, thereby regulating complement activation. This regulation is crucial for preventing excessive inflammation and tissue damage . Additionally, SERPING1 is involved in other physiological pathways, including blood coagulation, fibrinolysis, and the generation of kinins .

A deficiency in SERPING1 is associated with a condition known as Hereditary Angioedema (HAE). This genetic disorder is characterized by recurrent episodes of severe swelling (angioedema) in various parts of the body, including the extremities, face, gastrointestinal tract, and airway . The deficiency can be due to mutations that lead to either reduced levels of the protein or the production of a dysfunctional protein.

The recombinant form of SERPING1, produced in Human Embryonic Kidney (HEK) cells, is used for therapeutic purposes. Recombinant technology allows for the production of large quantities of the protein with high purity and activity. This recombinant protein is used in the treatment of HAE to replace the deficient or dysfunctional C1 inhibitor in patients .

Research on SERPING1 continues to explore its broader implications in various physiological and pathological processes. Understanding its role in the complement system and other pathways can lead to the development of new therapeutic strategies for diseases related to immune dysregulation, coagulation disorders, and inflammatory conditions .