SERPING1 Human
Description
Product Specs
Product Science Overview
The SERPING1 gene is located on chromosome 11 and encodes a highly glycosylated plasma protein . The protein is synthesized in the liver and is involved in the regulation of the complement cascade by inhibiting activated C1r and C1s of the first complement component . This inhibition is essential for controlling the activation of the complement system, which is a part of the immune response.
The primary function of C1 Inhibitor is to form a proteolytically inactive stoichiometric complex with the C1r or C1s proteases . This action prevents the uncontrolled activation of the complement system, which could otherwise lead to tissue damage and inflammation. Additionally, C1 Inhibitor is a very efficient inhibitor of FXIIa, chymotrypsin, and kallikrein .
Deficiency or dysfunction of C1 Inhibitor is associated with a condition known as hereditary angioedema (HAE) . HAE is characterized by recurrent episodes of severe swelling (angioedema) in various parts of the body, including the extremities, face, gastrointestinal tract, and airway. This condition can be life-threatening if it leads to airway obstruction.
Human recombinant C1 Inhibitor is produced using recombinant DNA technology, which allows for the production of the protein in a controlled environment. This recombinant form is used therapeutically to treat patients with HAE by providing a functional C1 Inhibitor to regulate the complement system and prevent angioedema attacks.
Research on SERPING1 and its encoded protein continues to expand our understanding of its role in various physiological processes and its potential therapeutic applications. The recombinant form of C1 Inhibitor is also being explored for its use in other conditions where complement activation plays a role, such as certain autoimmune diseases and inflammatory conditions.
In summary, Serpin Peptidase Inhibitor, Clade G Member 1 (Human Recombinant) is a vital protein with significant roles in immune regulation and clinical applications, particularly in the treatment of hereditary angioedema.
