C1-inhibitor
C1-Inhibitor
C1-Inhibitor (pronounced as "see one inhibitor") is a protein that plays a crucial role in regulating the complement system, a part of the immune system. It is also involved in the regulation of other biological pathways such as the coagulation system and the fibrinolytic system.
Etymology
The term "C1-Inhibitor" is derived from its function. It inhibits the first component (C1) of the complement system, hence the name C1-Inhibitor.
Function
C1-Inhibitor is a serine protease inhibitor (serpin) that regulates several key biological pathways. It inhibits the C1r and C1s proteases of the C1 complex of the complement system, thereby preventing the activation of this system. It also inhibits other proteases such as Factor XII and kallikrein in the coagulation system and the fibrinolytic system, respectively.
Clinical significance
Deficiency or dysfunction of C1-Inhibitor can lead to a rare condition known as Hereditary Angioedema (HAE). This condition is characterized by recurrent episodes of severe swelling (angioedema) in various parts of the body. Treatment for HAE often involves replacement therapy with C1-Inhibitor.
Related terms
- Complement system
- Coagulation system
- Fibrinolytic system
- Hereditary Angioedema
- Protein
- Serine protease inhibitor
- Factor XII
- Kallikrein
External links
- Medical encyclopedia article on C1-inhibitor
- Wikipedia's article - C1-inhibitor
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