Sherman L A, Gaston L W, Kaplan M E, Spivack A R
J Clin Invest. 1972 Mar;51(3):590-7. doi: 10.1172/JCI106848.
A patient with classical hemophilia (factor VIII deficiency) was found to have a new abnormal fibrinogen (fibrinogen St. Louis). Other family members exhibited either defect alone. Fibrinogen St. Louis was inherited as an autosomal dominant and was not associated with clinical bleeding. When compared with normal fibrinogen, fibrinogen St. Louis was found to have defective fibrin polymerization and possibly a slower release of fibrinopeptides. The prolonged thrombin times were partially corrected by calcium chloride and protamine sulfate. Ultracentrifugal sedimentation, electrophoretic mobility, DEAE chromatographic pattern, carbohydrate content, N-terminal amino acids, immunodiffusion, and immunoelectrophoretic patterns and electrophoresis of reduced and alkylated fragments were all normal. In contrast to fibrinogen St. Louis, the most similar other fibrinogen variant (fibrinogen Zurich) was found to be heterogeneous by several criteria and to have reduced hexose content.
一名患有典型血友病（因子 VIII 缺乏症）的患者被发现有一种新的异常纤维蛋白原（纤维蛋白原圣路易斯）。其他家庭成员单独表现出要么是因子 VIII 缺乏，要么是纤维蛋白原异常。纤维蛋白原圣路易斯以常染色体显性方式遗传，且与临床出血无关。与正常纤维蛋白原相比，发现纤维蛋白原圣路易斯的纤维蛋白聚合存在缺陷，并且纤维蛋白肽的释放可能较慢。凝血酶时间延长可被氯化钙和硫酸鱼精蛋白部分纠正。超速离心沉降、电泳迁移率、二乙氨基乙基纤维素色谱图谱、碳水化合物含量、N 端氨基酸、免疫扩散、免疫电泳图谱以及还原和烷基化片段的电泳均正常。与纤维蛋白原圣路易斯相反，通过多种标准发现最相似的其他纤维蛋白原变体（纤维蛋白原苏黎世）具有异质性且己糖含量降低。
