Patterson W R, Estry D W, Schwartz K A, Borchert R D, Bell T G
Department of Clinical Investigation, Wilford Hall USAF Medical Center, San Antonio, TX.
Thromb Haemost. 1989 Nov 24;62(3):1011-5.
Platelets from dogs with Basset Hound Hereditary Thrombopathy (BHT) initially displayed a thrombasthenia-like aggregation defect but have been shown to have normal amounts of platelet membrane glycoproteins IIb and IIIa (GPIIb-IIIa), and therefore are more accurately described as thrombopathic. The presence of normal quantities of GPIIb-IIIa, however, did not rule out the possibility of a functionally abnormal glycoprotein complex which would be unable to bind radio-labeled fibrinogen. Therefore, fibrinogen binding in BHT platelets was evaluated. Platelets from BHT and normal dogs were activated with 1 x 10(-5) M ADP in the presence of 125I-fibrinogen and the surface-bound radioactivity was quantitated. The amount of fibrinogen bound by BHT dog platelets was not significantly different than that bound by normal dog platelets. Platelets from dogs with BHT bound 30,282 +/- 3,133 and normal dog platelets bound 31,664 +/- 2,772 molecules of fibrinogen per platelet. The quantitatively normal GPIIb-IIIa complex binds fibrinogen in normal amounts and does not seem to represent the abnormality responsible for the aggregation defect in BHT platelets. Therefore, other factors central to normal platelet function and related to platelet aggregation must be considered.
患有巴吉度猎犬遗传性血小板病（BHT）的犬只的血小板最初表现出类似血小板无力症的聚集缺陷，但已证明其血小板膜糖蛋白IIb和IIIa（GPIIb-IIIa）含量正常，因此更准确地描述为血小板病性的。然而，正常量的GPIIb-IIIa的存在并不排除存在功能异常的糖蛋白复合物的可能性，这种复合物可能无法结合放射性标记的纤维蛋白原。因此，对BHT血小板中的纤维蛋白原结合情况进行了评估。在125I-纤维蛋白原存在的情况下，用1×10（-5）M ADP激活来自BHT犬和正常犬的血小板，并对表面结合的放射性进行定量。BHT犬血小板结合的纤维蛋白原量与正常犬血小板结合的纤维蛋白原量无显著差异。BHT犬的血小板每血小板结合30,282±3,133个纤维蛋白原分子，正常犬的血小板每血小板结合31,664±2,772个纤维蛋白原分子。定量正常的GPIIb-IIIa复合物以正常量结合纤维蛋白原，似乎并不代表导致BHT血小板聚集缺陷的异常因素。因此，必须考虑正常血小板功能中与血小板聚集相关的其他核心因素。
