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一种新的血小板特异性抗原Groa,定位于糖蛋白IIIa上,与新生儿同种免疫性血小板减少症有关。

A new private platelet antigen, Groa, localized on glycoprotein IIIa, involved in neonatal alloimmune thrombocytopenia.

作者信息

Simsek S, Vlekke A B, Kuijpers R W, Goldschmeding R, von dem Borne A E

机构信息

Central Laboratory, Red Cross Blood Transfusion Service, Amsterdam, The Netherlands.

出版信息

Vox Sang. 1994;67(3):302-6. doi: 10.1111/j.1423-0410.1994.tb01256.x.

DOI:10.1111/j.1423-0410.1994.tb01256.x
PMID:7863631
Abstract

The serum of a Caucasian woman who gave birth to a child with neonatal alloimmune thrombocytopenia contained antibodies directed against a platelet antigen of the newborn. There was no incompatibility for the known platelet alloantigens HPA-1 to HPA-7 or for the private or low-frequency antigens Sra and Vaa, between the platelets of the parents. However, crossmatching with the serum of the mother and the platelets of the child and the father was strongly positive, suggesting a new platelet antibody specificity. To investigate the inheritance of the 'Groa' antigen involved, the available family members were tested in the platelet immunofluorescence test (PIFT) and the monoclonal antibody-specific immobilization of platelet antigens (MAIPA) assay. The Groa antigen was found to be inherited in an autosomal-codominant fashion. In the MAIPA, we localized the Groa antigen on the glycoprotein IIb/IIIa complex (alpha IIb beta 3). The GP IIb/IIIa localization was confirmed in immunoprecipitation studies. In Western blotting experiments, we further localized the Groa antigen on the GP IIIa (beta 3) subunit of the GP IIb/IIIa complex. Until now we have tested approximately 400 unrelated donors. None of these appeared to be positive for the Groa antigen, suggesting a phenotype frequency in the Dutch population of less than 0.01.

摘要

一名白种女性分娩出患有新生儿同种免疫性血小板减少症的婴儿,其血清中含有针对新生儿血小板抗原的抗体。父母的血小板之间,对于已知的血小板同种抗原HPA - 1至HPA - 7以及私有或低频抗原Sra和Vaa均不存在不相容性。然而,母亲血清与孩子及父亲的血小板进行交叉配型呈强阳性,提示存在一种新的血小板抗体特异性。为研究所涉及的“Groa”抗原的遗传方式,对现有的家庭成员进行了血小板免疫荧光试验(PIFT)和血小板抗原单克隆抗体特异性固定试验(MAIPA)检测。发现Groa抗原以常染色体共显性方式遗传。在MAIPA试验中,我们将Groa抗原定位在糖蛋白IIb/IIIa复合物(αIIbβ3)上。免疫沉淀研究证实了GP IIb/IIIa的定位。在蛋白质免疫印迹实验中,我们进一步将Groa抗原定位在GP IIb/IIIa复合物的GP IIIa(β3)亚基上。到目前为止,我们已经检测了大约400名无关供者。这些人中没有一个Groa抗原呈阳性,这表明荷兰人群中该表型的频率低于0.01。

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