Hessel B, Stenbjerg S, Dyr J, Kudryk B, Therkildsen L, Blombäck B
Thromb Res. 1986 Apr 1;42(1):21-37. doi: 10.1016/0049-3848(86)90193-3.
Fibrinogen Aarhus was found in a woman with slightly prolonged whole blood clotting time. The thrombin induced clotting of plasma and purified fibrinogen was much prolonged. Kinetic analysis of FPA and FPB release revealed larger apparent Km and Vmax values for fibrinogen Aarhus than for normal fibrinogen. No clot formation of fibrinogen Aarhus was demonstrated in the presence of Batroxobin and the release of FPA was slower than normal. Upon addition of the clotting enzyme from Agkistrodon contortrix contortrix clotting did occur but the clotting time was much prolonged in comparison with normal fibrinogen. The turbidity of fibrin gels obtained from fibrinogen Aarhus was similar to normal fibrin gels at low thrombin concentrations. Increasing thrombin concentration resulted in appearance of degradation products in the fibrin gels from fibrinogen Aarhus and at the same time a relative increase in turbidity of the gels was observed. Possibly reasons for the slow release of fibrinopeptides, the delayed gelation, and susceptibility to degradation by thrombin are discussed.
在一名全血凝固时间稍有延长的女性体内发现了奥胡斯纤维蛋白原。凝血酶诱导的血浆和纯化纤维蛋白原的凝固时间大大延长。对纤维蛋白肽A(FPA)和纤维蛋白肽B(FPB)释放的动力学分析显示,与正常纤维蛋白原相比,奥胡斯纤维蛋白原的表观米氏常数(Km)和最大反应速度(Vmax)值更大。在巴曲酶存在的情况下,未观察到奥胡斯纤维蛋白原形成凝块,且FPA的释放比正常情况慢。加入五步蛇毒凝血酶后确实发生了凝血,但与正常纤维蛋白原相比,凝血时间大大延长。在低凝血酶浓度下,从奥胡斯纤维蛋白原获得的纤维蛋白凝胶的浊度与正常纤维蛋白凝胶相似。凝血酶浓度增加导致奥胡斯纤维蛋白原的纤维蛋白凝胶中出现降解产物,同时观察到凝胶浊度相对增加。文中讨论了纤维蛋白肽释放缓慢、凝胶化延迟以及对凝血酶降解敏感的可能原因。