Serpin Peptidase Inhibitor, Clade G Member 1, HEK Protein

Background

SERPING1, also known as C1 esterase inhibitor (C1-INH), is a serine protease inhibitor that plays a critical role in regulating the complement system, a part of the immune response. SERPING1 is primarily responsible for controlling the activation of the C1 complex in the classical pathway of complement activation. Beyond complement regulation, it also inhibits several other proteases involved in the coagulation, fibrinolytic, and kinin-generating systems. SERPING1 mutations or deficiencies are strongly linked to hereditary angioedema (HAE), a potentially life-threatening condition characterized by uncontrolled swelling. SERPING1 is an important therapeutic target in diseases where inappropriate immune activation or excessive protease activity leads to tissue damage or inflammation. It is mainly synthesized in the liver and secreted into the bloodstream, where it circulates and exerts its regulatory effects.