McDonagh J, McDonagh R P, Delâge J M, Wagner R H
J Clin Invest. 1969 May;48(5):940-6. doi: 10.1172/JCI106053.
Plasma and platelet factor XIII levels were measured in normal human donors and in a patient congenitally deficient in factor XIII. The purpose of these experiments was to study the role of platelet factor XIII in blood coagulation. On polyacrylamide disc electrophoresis, factor XIII activity in extracts of washed normal platelets appeared as a single peak. This peak was missing or very low when factor XIII-deficient platelet extract was used. The patient was also studied before and after transfusion of fresh frozen plasma. Before transfusion, factor XIII activity could not be detected in the patient's plasma or platelet extracts. 24 hr after transfusion the plasma factor XIII level was still at the anticipated value, but factor XIII activity could not be detected in the platelet extracts. These experiments imply that plasma factor XIII was not transported across the platelet membrane in measurable quantities in vivo. This suggests that platelet factor XIII is a true platelet component and originates in the platelet precursor, the megakaryocyte. Thrombelastographic studies suggest that platelet factor XIII participates in the coagulation process. Thrombelastograms of factor XIII-deficient samples had decreased maximum amplitude and clot elasticity values. The abnormalities were fully corrected by the addition of washed normal platelets to factor XIII-deficient plasma; preincubation of the normal platelets in the deficient plasma had no additional effect. This indicates that platelet factor XIII is immediately available during clot formation.
在正常人体供体和一名先天性缺乏因子 XIII 的患者中测量了血浆和血小板因子 XIII 水平。这些实验的目的是研究血小板因子 XIII 在血液凝固中的作用。在聚丙烯酰胺圆盘电泳中,洗涤后的正常血小板提取物中的因子 XIII 活性表现为一个单一峰。当使用缺乏因子 XIII 的血小板提取物时,这个峰缺失或非常低。还对该患者在输注新鲜冷冻血浆前后进行了研究。输血前,在患者的血浆或血小板提取物中未检测到因子 XIII 活性。输血后 24 小时,血浆因子 XIII 水平仍处于预期值,但在血小板提取物中未检测到因子 XIII 活性。这些实验表明,血浆因子 XIII 在体内不会以可测量的量穿过血小板膜。这表明血小板因子 XIII 是真正的血小板成分,起源于血小板前体巨核细胞。血栓弹力图研究表明血小板因子 XIII 参与凝血过程。缺乏因子 XIII 的样本的血栓弹力图的最大振幅和血凝块弹性值降低。通过向缺乏因子 XIII 的血浆中添加洗涤后的正常血小板,这些异常得到了完全纠正;在缺乏因子 XIII 的血浆中对正常血小板进行预孵育没有额外影响。这表明血小板因子 XIII 在血凝块形成过程中可立即发挥作用。