Hofmann V, Gati W P, Straub P W
Thromb Haemost. 1979 Jun 30;41(4):709-13.
Fibrinogen Zurich I is characterized by an abnormal fibrin monomer polymerization. It consists of two fractions of molecules, one with a normal aggregation and one not aggregating at all and interfering with the aggregation of the normal population. Using a radioimmunoassay for fibrinopeptide A, only approximately half of the expected fibrinopeptide A could be recovered after thrombin or Defibrase proteolysis. The defective fibrinopeptide A release could be confirmed by measurement of the N-terminal Gly/Tyr ratio. It is likely that the abnormal fibrin monomer aggregation of the abnormal fraction of fibrinogen Zurich I is due to the defective fibrinopeptide A release of this fraction.
苏黎世纤维蛋白原I的特征是纤维蛋白单体聚合异常。它由两部分分子组成,一部分具有正常聚集性,另一部分完全不聚集并干扰正常群体的聚集。使用针对纤维蛋白肽A的放射免疫测定法,在凝血酶或去纤维蛋白酶蛋白水解后,只能回收大约一半预期的纤维蛋白肽A。通过测量N端甘氨酸/酪氨酸比率可以证实纤维蛋白肽A释放存在缺陷。苏黎世纤维蛋白原I异常部分的异常纤维蛋白单体聚集很可能是由于该部分纤维蛋白肽A释放存在缺陷所致。