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以色列伊拉克犹太人和阿拉伯人群中的I型Glanzmann血小板无力症患者可通过血小板糖蛋白IIIa免疫印迹分析加以区分。

Type I Glanzmann thrombasthenia patients from the Iraqi-Jewish and Arab populations in Israel can be differentiated by platelet glycoprotein IIIa immunoblot analysis.

作者信息

Coller B S, Seligsohn U, Little P A

出版信息

Blood. 1987 Jun;69(6):1696-703.

PMID:3580574
Abstract

A sensitive immunoblot technique for platelet glycoprotein IIIa (GPIIIa) was used to analyze the platelets of patients living in Israel who meet the diagnostic criteria for type I Glanzmann thrombasthenia. When reacted with solubilized normal platelets, a rabbit antiserum to GPIIIa identified a major band at molecular weight (mol wt) 90,000 and three additional minor bands at Mr 110,000, 81,000, and 64,000. The major band could not be detected, and the minor bands were either markedly reduced or absent in the platelet samples from 14 of the 15 patients from the Iraqi-Jewish population. In contrast, in all four Arab patients tested, the major band was detectable, although at markedly reduced levels, and the minor bands were either markedly reduced or absent; an additional minor band at mol wt 47,000 was also present in the platelets from these patients. One Iraqi-Jewish patient had a unique pattern in which two of the bands were present but reduced and two were undetectable. We conclude that the protein defect, and thus presumably the genetic defect, causing Glanzmann thrombasthenia in the majority of patients in the Iraqi-Jewish population differs from that in the Arab population, and we confirm that there is considerable biochemical heterogeneity among the patients who meet the criteria for type I Glanzmann thrombasthenia.

摘要

采用一种针对血小板糖蛋白IIIa(GPIIIa)的灵敏免疫印迹技术,对符合I型Glanzmann血小板无力症诊断标准的以色列患者的血小板进行分析。当与溶解的正常血小板反应时,一种针对GPIIIa的兔抗血清识别出一条分子量(mol wt)为90,000的主要条带以及另外三条分子量分别为110,000、81,000和64,000的次要条带。在来自伊拉克犹太人群体的15名患者中的14名患者的血小板样本中,无法检测到主要条带,次要条带要么明显减少要么缺失。相比之下,在所有4名接受检测的阿拉伯患者中,虽然主要条带水平明显降低但仍可检测到,次要条带要么明显减少要么缺失;这些患者的血小板中还存在一条分子量为47,000的额外次要条带。一名伊拉克犹太患者呈现出一种独特的模式,其中两条条带存在但减少,另外两条无法检测到。我们得出结论,导致伊拉克犹太人群体中大多数患者出现Glanzmann血小板无力症的蛋白质缺陷以及推测的基因缺陷与阿拉伯人群体中的不同,并且我们证实,符合I型Glanzmann血小板无力症标准的患者之间存在相当大的生化异质性。

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