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[Glanzmann血小板无力症和Bernard-Soulier综合征患者血小板中蛋白质异常的免疫化学研究]

[Immunochemical study of protein anomalies in platelets of patients with Glanzmann's thrombasthenia and Bernard-Soulier syndrome].

作者信息

Nurden A T, Hagen I, Kunicki T, Caen J P

出版信息

C R Seances Acad Sci D. 1979 Jul 9;289(2):209-11.

PMID:117927
Abstract

Crossed immunoelectrophoresis of "Triton X-100" solubilized proteins from (125I) iodinated normal and abnormal (Bernard-Soulier and thrombasthenic) platelets was performed using rabbit antibodies raised against whole, normal platelets. In Bernard-Soulier platelets neither of the two surface oriented forms of GP I, glycocalicin and glycoprotein Ib (band 13) were detected. In two patients with type I thrombasthenia, the most prominent precipitate (No. 16) and platelet fibrinogen (No 24) were not detected whereas in one patient with type II thromblasthenia, fibrinogen was normally detected and the amount of protein 16 was around 15% of the normal level. The alloantibody IgG L..., able to induce a "thrombasthenic-like" reactivity in normal human platelets, has been shown to be directed against protein 16.

摘要

使用针对完整正常血小板产生的兔抗体,对来自(125I)碘化正常及异常(伯纳德-索利尔综合征和血小板无力症)血小板的“曲拉通X-100”增溶蛋白进行交叉免疫电泳。在伯纳德-索利尔综合征血小板中,未检测到糖蛋白I的两种表面定向形式,即糖萼蛋白和糖蛋白Ib(第13条带)。在两名I型血小板无力症患者中,未检测到最显著的沉淀(第16号)和血小板纤维蛋白原(第24号),而在一名II型血小板无力症患者中,正常检测到纤维蛋白原,且蛋白16的量约为正常水平的15%。已证明能在正常人血小板中诱导“类血小板无力症”反应性的同种抗体IgG L... 针对蛋白16。

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