Acquired Bernard-Soulier syndrome. Evidence for the role of a 210,000-molecular weight protein in the interaction of platelets with von Willebrand factor.
Acquired Bernard-Soulier syndrome. Evidence for the role of a 210,000-molecular weight protein in the interaction of platelets with von Willebrand factor.
复制标题
获得性伯纳德-苏利埃综合征。
DOI：
10.1172/jci112084
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发表时间：
1985
期刊：
影响因子：
--
通讯作者：
Shuman,MA
中科院分区：
文献类型：
--
作者：
Stricker,RB;Wong,D;Saks,SR;Corash,L;Shuman,MA
一例淋巴细胞增生性疾病患者在利斯托司汀治疗后出现出血，出血时间延长，选择性血小板聚集缺陷。患者纯化的IgG被证明可以抑制利斯托司汀和血管性血变因子（fviii:vWF）对洗涤后的正常血小板的聚集。Western blotting结果显示，在先天性Bernard-Soulier综合征（BSS）患者的血小板中，存在特异性结合Mr 210,000抗原的抗体，但在血小板中缺失。结合受到IgG的F(ab)2部分的影响，表明存在自身抗体而不是免疫复合物。这些结果表明210,000-Mr蛋白参与了fviii:vWF与血小板的相互作用。此外，我们已经证明先天性BSS血小板明显缺乏额外的蛋白质。热聚集IgG也被证明与210,000-Mr蛋白结合，表明该蛋白可能作为血小板上的Fc受体起作用。210,000-Mr蛋白与糖蛋白Ib的关系，以及该蛋白在血小板与fviii:vWF相互作用中的确切作用，需要进行表征。图片
A patient with a lymphoproliferative disorder developed bleeding associated with a prolonged bleeding time and a selective defect of platelet aggregation in response to ristocetin. The patient's purified IgG was shown to inhibit aggregation of washed normal platelets by ristocetin and von Willebrand factor (F VIII:vWF). By Western blotting, it was shown that antibody bound specifically to an antigen of Mr 210,000 present on normal platelets but missing on platelets from patients with congenital Bernard-Soulier syndrome (BSS). Binding was effected by the F(ab)2 portion of the IgG, indicating the presence of an autoantibody rather than an immune complex. These results suggest that the 210,000-Mr protein is involved in the interaction of F VIII:vWF with platelets. Furthermore, we have demonstrated the apparent absence of an additional protein on congenital BSS platelets. Heat-aggregated IgG was also shown to bind to the 210,000-Mr protein, suggesting that this protein may function as an Fc receptor on platelets. The relationship of the 210,000-Mr protein to glycoprotein Ib and the precise role of this protein in the interaction of platelets with F VIII:vWF need to be characterized.Images