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DOI: 10.1055/s-0037-1622007
Die Rolle von Faktor XIII bei kardiound zerebrovaskulären Erkrankungen
Role of coagulation factor XIII in cardioand cerebrovascular diseasesPublikationsverlauf
Publikationsdatum:
29. Dezember 2017 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00034925/200201/lookinside/thumbnails/10-1055-s-0037-1622007_de-1.jpg)
Zusammenfassung
Am Ende der Gerinnungskaskade aktiviert Thrombin den Blutgerinnungsfaktor XIII (FXIII). Das aktivierte Enzym (FXIIIa) überführt lösliches Fibrin in quervernetztes, unlösliches Fibrin. Die Rolle von FXIII bei der Entstehung kardio- und zerebrovaskulärer Erkrankungen wird erst seit kurzem untersucht. Der häufig vorkommende FXIII-Val34Leu-Polymorphismus im Gen der FXIII-A-Untereinheit hat zwar einen protektiven Effekt gegen Herz- und ischämischen Hirninfarkt, ist aber auch mit einem erhöhten Risiko für hämorrhagischen zerebrovaskulären Insult verbunden. Vermutlich schützt FXIII-Val34Leu auch vor Lungenembolie und tiefer Beinvenenthrombose. Als mögliche Mechanismen werden die vorzeitige Elimination des mutierten FXIIIa aus der Zirkulation sowie veränderte Strukturen der durch den mutierten FXIIIa vernetzten Gerinnsel diskutiert. Auch aufgrund der Interaktion zwischen FXIII und dem Insulinresistenzsyndrom, also der Wechselwirkung zwischen Komponenten der Blutgerinnung und klassischen metabolischen atheromatösen Risikofaktoren, muss FXIII als weiterer Gerinnungsfaktor betrachtet werden, der zu den komplexen Gen-Umwelt-Wechselwirkungen beiträgt, denen pathogenetische Bedeutung bei kardio- und zerebrovaskulären sowie thromboembolischen Erkrankungen zukommt.
Summary
In the final step of the clotting cascade coagulation factor XIII (FXIII) is activated by thrombin. The activated enzyme (FXIIIa) has an important role in the final stage of blood coagulation in cross-linking soluble fibrin to a stable insoluble clot. The role of FXIII in cardio- and cerebrovascular diseases has been investigated recently. The widespread Val34Leu polymorphism in the gene coding for the FXIII subunit A (FXIII Val34Leu) has been shown to protect against myocardial infarction and ischemic stroke, but is also associated with an increased risk for hemorrhagic stroke. Additionally, FXIII Val34Leu is supposed to be protective against pulmonary embolism and deep vein thrombosis. As possible mechanisms for the antithrombotic effect, premature depletion of the mutant protein from circulation and altered fibrin structures of clots cross-linked by the mutant FXIIIa are under discussion. The connection between FXIII and the insulin resistance syndrome also attracts attention, i. e. the interaction between a component of the coagulation cascade and thus a thrombotic risk factor and classical atheromatous risk factors. Therefore, FXIII must be considered as another coagulation factor contributing to complex interactions between genes and environment important for the pathogenesis of cardio- and cerebrovascular and thromboembolic diseases.
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