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Published in: Clinical Reviews in Allergy & Immunology 3/2021

Open Access 01-06-2021 | Angioedema

Blood Clotting and the Pathogenesis of Types I and II Hereditary Angioedema

Authors: Steven de Maat, Kusumam Joseph, Coen Maas, Allen P. Kaplan

Published in: Clinical Reviews in Allergy & Immunology | Issue 3/2021

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Abstract

The plasma contact system is the initiator of the intrinsic pathway of coagulation and the main producer of the inflammatory peptide bradykinin. When plasma is exposed to a negatively charged surface the two enzymes factor XII (FXII) and plasma prekallikrein (PK) bind to the surface alongside the co-factor high molecular weight kininogen (HK), where PK is non-covalently bound to. Here, FXII and PK undergo a reciprocal activation feedback loop that leads to full contact system activity in a matter of seconds. Although naturally occurring negatively charged surfaces have shown to be involved in the role of the contact system in thrombosis, such surfaces are elusive in the pathogenesis of bradykinin-driven hereditary angioedema (HAE). In this review, we will explore the molecular mechanisms behind contact system activation, their assembly on the endothelial surface, and their role in the HAE pathophysiology.
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Metadata
Title
Blood Clotting and the Pathogenesis of Types I and II Hereditary Angioedema
Authors
Steven de Maat
Kusumam Joseph
Coen Maas
Allen P. Kaplan
Publication date
01-06-2021
Publisher
Springer US
Published in
Clinical Reviews in Allergy & Immunology / Issue 3/2021
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI
https://doi.org/10.1007/s12016-021-08837-6

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