Cat# : THP-0016
|Product Name:||Native Human C1-esterase inhibitor (hC1INH)|
|Description:||The product is composed of purified endogenous complement component-1 esterase inhibitor (hC1INH) isolated from human plasma. The primary function of endogenous C1INH is to regulate the activation of the complement and contact system pathways. It does this through inhibition of several target proteases within these pathways including activated C1s, kallikrein, factor XIIa and factor XIa. C1 esterase inhibitor has also been shown to inhibit the action of thrombin within the coagulation pathway, and tPA and plasmin within the fibrinolytic pathway. Deficiency of C1-inhibitor permits plasma kallikrein activation, which leads to the production of the vasoactive peptide bradykinin. Additionally, C4 and C2 cleavage goes unchecked, resulting in auto-activation of the complement system. Down-stream effects of the lack of enzyme inhibition by C1 esterase inhibitor results in swelling due to leakage of fluid from blood vessels into connective tissue and consequently the presentation of hereditary angioedema (HAE).|
|Molecular Weight:||68000.0 Da|
|Cas No:||Not Available|
|Endotoxin Level:||<0.001 EU per 1 μg by the LAL method|
|Drug Name:||Human C1-esterase inhibitor|
|Applications:||For routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE).|
|Examples of Clinical Use:||Angioedema attacks in Hereditary Angioedema (HAE).|
|Pharmacodynamics:||In clinical studies, intravenous administration demonstrated an increase in plasma levels of C1 inhibitor within approximately one hour or less of administration. Biological activity was shown in 35 subjects by the subsequent increase in plasma C4 levels from an average of C4 8.1 mg/mL at baseline to C4 8.6 mg/mL 12 hours after infusion.|
|Mechanism of action:||C1 inhibitor is a normal constituent of human blood and is one of the serine proteinase inhibitors (serpins). The primary function of C1 inhibitor is to regulate the activation of the complement and intrinsic coagulation (contact system) pathway. C1 inhibitor also regulates the fibrinolytic system. Regulation of these systems is performed through the formation of complexes between the proteinases and the inhibitor, resulting in inactivation of both and consumption of the C1 inhibitor. HAE patients have low levels of endogenous or functional C1 inhibitor. Although the events that induce attacks of angioedema in HAE patients are not well defined, it is thought by some that increased vascular permeability and the clinical manifestation of HAE attacks are primarily mediated through contact system activation. Suppression of contact system activation by C1 inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin1. Administration of C1 Esterase Inhibitor increases plasma levels of C1 inhibitor activity.|
|Affected organisms:||Humans and other mammals|
|Targets:||Target 1. Complement C1r subcomponent; Target 2. Complement C1s subcomponent; Target 3. Plasma kallikrein; Target 4. Coagulation factor XII; Target 5. Prothrombin; Target 6. Coagulation factor XI; Target 7. Tissue-type plasminogen activator|
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