2010
DOI: 10.1016/j.jaci.2010.08.012
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Enzymatic pathways in the pathogenesis of hereditary angioedema: The role of C1 inhibitor therapy

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Cited by 99 publications
(78 citation statements)
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“…Of these, the contact system is generally considered the most important for the pathogenesis of the angioedema attacks [5,6].…”
Section: Introductionmentioning
confidence: 99%
“…Of these, the contact system is generally considered the most important for the pathogenesis of the angioedema attacks [5,6].…”
Section: Introductionmentioning
confidence: 99%
“…C1-Inh is a 110-kDa glycoprotein that regulates activation of the complement cascade and fibrinolysis [11]. Its main function is to inhibit the C1r, C1s, MASP-1 and MASP-2 proteases and halt activation of the cascade.…”
Section: Discussionmentioning
confidence: 99%
“…In addition, it functions to inhibit proteases of the contact system (plasma kallikrein and activated Hageman factor XIIa and XIIf), which are responsible for the clinical course in HAE. Finally, it exerts inhibitory effects on plasmin and protease Xia (coagulation pathways) [11]. It is considered a suicide protein whose mechanism of action is to form 1:1 stoichiometric complexes with the proteases, thus allowing its clearance and inactivation [11].…”
Section: Discussionmentioning
confidence: 99%
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“…Increased bradykinin levels increase vascular permeability and extravasation, manifesting as edema (Figure 2)[1,28-30]. This is an important differentiating feature of HAE when compared with allergic reactions, which are primarily mediated by histamine.…”
Section: Clinical Presentationmentioning
confidence: 99%