What is C1 esterase function?
Clinical Information C1 inhibitor (C1-INH) is a multispecific protease inhibitor that is present in normal human plasma and serum, and which regulates enzymes of the complement, coagulation, fibrinolytic, and kinin-forming systems.
What does a high C1 esterase inhibitor mean?
If your C1-INH levels are lower or higher than normal, it may be a sign of: hereditary or acquired angioedema. SLE. kidney diseases, such as lupus nephritis, glomerulonephritis, or membranous nephritis. septicemia, which is also known as blood infection.
What is the substrate for C1 esterase?
Although its name suggests specificity for C1, C1-INH acts on a variety of substrates including the contact system proteases (factor XII, plasma kallikrein), an intrinsic coagulation protease (factor XI) and the fibrinolytic proteases (plasmin, tissue plasminogen activator).
What causes C1 deficiency?
Acquired C1-inhibitor deficiency and B-cell disorders The main causes of acquired C1-inhibitor deficiency are lymphoproliferative disease and autoimmune disease.
How does C1 esterase deficiency cause angioedema?
C1 esterase inhibitor deficiency, also known as hereditary angioedema, results in the unchecked production of the vasodilator bradykinin.
How does C1 esterase deficiency lead to angioedema?
What is C1 deficiency?
Introduction. Hereditary angioedema, otherwise known as C1 esterase deficiency, is defined by recurrent episodes of angioedema without urticaria or pruritus. These skin conditions typically involve the legs, hands, face, upper respiratory tract, as well as gastrointestinal tract.
What causes Acquired angioedema?
Causes. Acquired angioedema occurs when levels of a protein called C1-inhibitor (C1-I) are too low. This protein blocks the activity of two other proteins; plasma kallikrein and coagulation factor 12. These two proteins are important for blood clotting, inflammation, and wound healing.
What does C1 mean in a blood test?
C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood. It controls a protein called C1, which is part of the complement system. The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells.
How are C1 esterase inhibitors used in medicine?
C1 esterase inhibitor is used to treat or prevent hereditary angioedema (HAE). HAE is a rare disease that causes swelling of the face, hands, feet, throat, stomach, bowels, or genitals. People who have HAE have low levels of C1 esterase inhibitor in their body. This medicine helps increase the amount of C1 esterase inhibitors in the body.
Can a C1 esterase inhibitor deficiency cause angioedema?
C1 Esterase inhibitor deficiency. (Hereditary Angioedema, HAE) HAE causes recurrent episodes of angioedema in the upper respiratory, gastrointestinal tract or in subcutaneous tissues.
Is there a blood test for esterase inhibitor?
Certain health problems can cause deficiencies in these proteins. Your doctor can order blood tests to check your complement protein levels. One of these tests is the C1 esterase inhibitor test or C1-INH test.
How are C1r and C1s proteases inactivated during inflammation?
The levels rise ~2-fold during inflammation. C1-inhibitor irreversibly binds to and inactivates C1r and C1s proteases in the C1 complex of classical pathway of complement. MASP-1 and MASP-2 proteases in MBL complexes of the lectin pathway are also inactivated.
