Surface modifications in the platelets of a patient with alpha-N-acetyl-D-galactosamine residues, the Tn-syndrome.
Surface modifications in the platelets of a patient with alpha-N-acetyl-D-galactosamine residues, the Tn-syndrome.
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DOI：
10.1172/jci110727
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发表时间：
1982-12
期刊：
影响因子：
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通讯作者：
A. Nurden;D. Dupuis;D. Pidard;N. Kieffer;T. Kunicki;J. Cartron
中科院分区：
文献类型：
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作者：
A. Nurden;D. Dupuis;D. Pidard;N. Kieffer;T. Kunicki;J. Cartron
Tn-综合征是一种获得性疾病，其特征在于血细胞的多凝集和细胞表面的α-N-乙酰基-D-半乳糖胺残基（Tn-抗原）的病理性暴露。我们现在报告了对一名患者（学士）血小板的研究。其中81%与苹果蠹凝集素（HPA）的荧光素结合物呈阳性反应。Ba.通过乳过氧化物酶催化的程序用125 I标记血小板;在十二烷基硫酸钠（SDS）-聚丙烯酰胺凝胶上的单向和双向电泳之后进行放射自显影，其显示主要膜糖蛋白（GP）的正常125 I标记，但GP Ib具有比正常更快的迁移。Ba.当用神经氨酸酶、半乳糖氧化酶和[3 H]硼氢化钠顺序处理血小板悬浮液时，血小板被强烈标记。与正常人血小板的GP Ib不同，当Ba。用半乳糖氧化酶和[3 H]硼氢化钠单独处理血小板。同种异体抗原PlA 1和奎尼丁依赖性抗体受体活性均由Ba正常表达。血小板，其也结合GP Ib的单克隆抗体（AN 51）。分析Ba。使用兔抗人血小板抗体制剂通过交叉免疫电泳检测血小板，揭示了在GP Ib位置存在免疫沉淀物，其具有异常的外观和在第二维中的迁移。还注意到由因子VIIIR：Ag给出的沉淀物的位置改变。在第一维电泳过程中，HPA掺入琼脂糖凝胶中导致Ba的异常GP Ib特异性沉淀。血小板我们的研究表明，循环Tn-血小板含有GP Ib与修饰的寡糖链结构负责Tn-抗原活性的血小板表达。
The Tn-syndrome is an acquired disorder characterized by the polyagglutination of blood cells and the pathological exposure of alpha-N-acetyl-D-galactosamine residues (Tn-antigen) at the cell surface. We now report studies on the platelet of a patient (Ba.) of which 81% reacted positively with a fluorescein conjugate of Helix pomatia agglutinin (HPA). The surface proteins of Ba. platelets were labeled with 125I by the lactoperoxidase-catalyzed procedure; single and two-dimensional electrophoresis on sodium dodecyl sulfate (SDS)-polyacrylamide gels was followed by autoradiography that revealed normal 125I-labeling of the major membrane glycoproteins (GP) but that GP Ib had a faster than normal migration. the abnormal GP Ib of Ba. platelets was strongly labeled when platelet suspensions were treated sequentially with neuraminidase, galactose oxidase, and sodium [3H]borohydride. Unlike the GP Ib of normal human platelets, it was also strongly labeled when Ba. platelets were treated with galactose oxidase and sodium [3H]borohydride alone. Both the alloantigen, PlA1, and quinidine-dependent antibody receptor activity were normally expressed by Ba. platelets, which also bound a monoclonal antibody (AN51) to GP Ib. Analysis of Ba. platelets by crossed immunoelectrophoresis using a rabbit anti-human platelet antibody preparation revealed the presence of an immunoprecipitate in the GP Ib position that had an abnormal appearance and migration in the second dimension. An altered position of the precipitate given by Factor VIIIR:Ag was also noted. Incorporation of HPA into the agarose gel during the first dimension electrophoresis resulted in the specific precipitation of the abnormal GP Ib of Ba. platelets. Our studies show that circulating Tn-platelets contain GP Ib with a modified oligosaccharide chain structure responsible for the platelet expression of Tn-antigen activity.