Muszbek L, Bagoly Z, Bereczky Z, Katona E
Clinical Research Center, Medical and Health Science Center, University of Debrecen, 98 Nagyerdei krt., P.O. Box 40, 4012 Debrecen, Hungary.
Cardiovasc Hematol Agents Med Chem. 2008 Jul;6(3):190-205. doi: 10.2174/187152508784871990.
It has been known for a long time that blood coagulation factor XIII (FXIII) is essential for maintaining haemostasis, its deficiency leads to severe bleeding complication. Biochemical studies have revealed that FXIII is a key regulator of fibrinolysis and, in addition to its role in haemostasis, it has also been implicated in the pathology of arterial and venous thrombosis. Most recently, the polymorphisms in the FXIII subunit genes and their influence on the risk of thrombotic diseases have stirred a lot of interest. This review, besides including the basic biochemistry of FXIII, mainly concentrates on the biochemical and clinical aspects of the involvement of FXIII in fibrinolysis and thrombosis. Biochemical aspects: Basics on the structure and activation of plasma and cellular FXIII. The enzymological features of activated FXIII and its main substrates. The interaction of FXIIIa with fibrinogen/fibrin and with components of the fibrinolytic system. The impact of cross-linked fibrin clot formation on the fibrinolytic processes. The down-regulation of FXIIIa within the fibrin clot. FXIII polymorphisms and their biochemical consequences. Clinical Aspects: FXIII level and the risk of arterial thrombosis (coronary artery disease, peripheral artery disease, ischemic stroke). The effect of FXIII subunit polymorphisms on the risk of arterial thrombotic diseases. The interplay between FXIII polymorphisms and other factors influencing the risk of arterial thrombosis. FXIII and venous thromboembolism.
长期以来，人们都知道凝血因子 XIII（FXIII）对于维持止血至关重要，其缺乏会导致严重的出血并发症。生化研究表明，FXIII 是纤维蛋白溶解的关键调节因子，除了在止血中的作用外，它还与动脉和静脉血栓形成的病理过程有关。最近，FXIII 亚基基因的多态性及其对血栓性疾病风险的影响引起了广泛关注。本综述除了介绍 FXIII 的基本生物化学外，主要集中在 FXIII 参与纤维蛋白溶解和血栓形成的生化及临床方面。生化方面：血浆和细胞 FXIII 的结构与激活的基础知识。活化 FXIII 的酶学特性及其主要底物。FXIIIa 与纤维蛋白原/纤维蛋白以及与纤维蛋白溶解系统成分的相互作用。交联纤维蛋白凝块形成对纤维蛋白溶解过程的影响。纤维蛋白凝块内 FXIIIa 的下调。FXIII 多态性及其生化后果。临床方面：FXIII 水平与动脉血栓形成风险（冠状动脉疾病、外周动脉疾病、缺血性中风）。FXIII 亚基多态性对动脉血栓性疾病风险的影响。FXIII 多态性与其他影响动脉血栓形成风险的因素之间的相互作用。FXIII 与静脉血栓栓塞。
