Mattson J C, Estry D W, Bell T G, Patterson W R
Thromb Res. 1986 Oct 1;44(1):23-38. doi: 10.1016/0049-3848(86)90177-5.
Basset Hound Hereditary Thrombopathy (BHT) is an autosomally inherited disorder of platelet function characterized by a thrombasthenia-like defect in aggregation but normal clot retraction. Glycoprotein IIb-IIIa (GP IIb-IIIa) is detectable in BHT platelets but may be functionally defective. In order to further characterize this potential model for human Glanzmann's thrombasthenia, contact reactivity of BHT platelets was studied by whole mount electron microscopy. Gel filtered BHT platelets, after 30 minutes of contact activation, attached poorly to a formvar substrate. There was an 8 fold difference in the number of adherent BHT platelets and normal platelets. In addition, contact induced shape change was inhibited when compared to control dogs. Almost 95% of control platelets reached fully dendritic or spread forms after 30 minutes of contact; in contrast only 63.7% of BHT platelets reached this degree of activation. The addition of 8.2 uM ADP to BHT platelets induced nearly a 4 fold increase in the number of spread forms and a 5 fold increase in the number of adherent BHT platelets, but did not cause aggregate formation. Both the defect in adhesion and shape change and the ability of ADP to stimulate both adhesion and contact-induced shape change in BHT platelets are similar to recent observations in our laboratory in patient's with type II Glanzmann's thrombasthenia.
巴吉度猎犬遗传性血小板病（BHT）是一种常染色体遗传性血小板功能障碍，其特征为聚集功能存在类血小板无力症样缺陷，但血块回缩正常。糖蛋白IIb-IIIa（GP IIb-IIIa）在BHT血小板中可检测到，但功能可能存在缺陷。为了进一步描述这种人类Glanzmann血小板无力症的潜在模型，通过整装电子显微镜研究了BHT血小板的接触反应性。凝胶过滤后的BHT血小板在接触激活30分钟后，与福尔马林中膜底物的附着较差。附着的BHT血小板数量与正常血小板数量存在8倍差异。此外，与对照犬相比，接触诱导的形态变化受到抑制。接触30分钟后，几乎95%的对照血小板达到完全树突状或伸展形态；相比之下，只有63.7%的BHT血小板达到这种激活程度。向BHT血小板中添加8.2 μM ADP可使伸展形态的数量增加近4倍，附着的BHT血小板数量增加5倍，但不会导致聚集形成。BHT血小板的黏附及形态变化缺陷以及ADP刺激BHT血小板黏附及接触诱导形态变化的能力，均与我们实验室近期对II型Glanzmann血小板无力症患者的观察结果相似。
