Factor XII Contact Activation
العنوان: | Factor XII Contact Activation |
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المؤلفون: | Thomas Renné, Stefan Blankenberg, Elena Burillo, Lynn M. Butler, Clément Naudin |
المصدر: | Seminars in Thrombosis and Hemostasis. 43:814-826 |
بيانات النشر: | Georg Thieme Verlag KG, 2017. |
سنة النشر: | 2017 |
مصطلحات موضوعية: | 0301 basic medicine, Conformational change, Factor XIIa, 030204 cardiovascular system & hematology, Protein aggregation, Bradykinin, Hereditary Angioedema Type III, 03 medical and health sciences, 0302 clinical medicine, Thrombin, Zymogen, medicine, Animals, Humans, Platelet, Blood Coagulation, Serine protease, Factor XII, biology, Chemistry, Hematology, Enzyme Activation, 030104 developmental biology, Coagulation, Biochemistry, biology.protein, Biophysics, Cardiology and Cardiovascular Medicine, medicine.drug |
الوصف: | Contact activation is the surface-induced conversion of factor XII (FXII) zymogen to the serine protease FXIIa. Blood-circulating FXII binds to negatively charged surfaces and this contact to surfaces triggers a conformational change in the zymogen inducing autoactivation. Several surfaces that have the capacity for initiating FXII contact activation have been identified, including misfolded protein aggregates, collagen, nucleic acids, and platelet and microbial polyphosphate. Activated FXII initiates the proinflammatory kallikrein-kinin system and the intrinsic coagulation pathway, leading to formation of bradykinin and thrombin, respectively. FXII contact activation is well characterized in vitro and provides the mechanistic basis for the diagnostic clotting assay, activated partial thromboplastin time. However, only in the past decade has the critical role of FXII contact activation in pathological thrombosis been appreciated. While defective FXII contact activation provides thromboprotection, excess activation underlies the swelling disorder hereditary angioedema type III. This review provides an overview of the molecular basis of FXII contact activation and FXII contact activation–associated disease states. |
تدمد: | 1098-9064 0094-6176 |
DOI: | 10.1055/s-0036-1598003 |
URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3a630d4eeb4cf815e807315478369856 https://doi.org/10.1055/s-0036-1598003 |
رقم الانضمام: | edsair.doi.dedup.....3a630d4eeb4cf815e807315478369856 |
قاعدة البيانات: | OpenAIRE |
تدمد: | 10989064 00946176 |
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DOI: | 10.1055/s-0036-1598003 |