Stroncek David F
Department of Transfusion Medicine, Clinical Center, National Institutes of Health, Bethesda, Maryland 20892, USA.
Curr Opin Hematol. 2007 Nov;14(6):688-93. doi: 10.1097/MOH.0b013e3282efed9e.
Several advances have been made in characterization of the molecule that carries human neutrophil antigen (HNA)-2a, NB1 glycoprotein, the gene that encodes NB1 glycoprotein, CD177, and the role of antibodies to HNA-2a in transfusion reactions.
NB1 glycoprotein binds to the endothelial cell adhesion molecule, platelet endothelial cell adhesion molecule-1 (PECAM-1), and participates in neutrophil transmigration. The overexpression of neutrophil CD177 mRNA has become a useful, but nonspecific biomarker of myeloproliferative diseases, especially polycythemia vera. CD177 mRNA overexpression is also a biomarker of a subset of patients with essential thrombocythemia who are at increased risk of thromboembolic complications. In patients with myeloproliferative disorders CD177 mRNA overexpression is secondary to a gain-of-function mutation in JAK2, JAK2 V617F. NB1 glycoprotein is co-localized on neutrophil plasma membranes with proteinase 3 and a complex of NB1 glycoprotein and proteinase 3 may initiate the activation of neutrophils by antineutrophil cytoplasmic antibodies in patients with Wegener's granulomatosis. The inadvertent transfusion of antibodies to HNA-2a with blood components frequently causes pulmonary transfusion reactions.
The expression of CD177 is an important biomarker of myeloproliferative diseases, NB1 glycoprotein is a ligand for PECAM-1 and it may have a role in Wegener's granulomatosis, and antibodies to HNA-2a frequently cause pulmonary transfusion reactions.
在携带人类嗜中性粒细胞抗原（HNA）-2a的分子（NB1糖蛋白）、编码NB1糖蛋白的基因（CD177）以及抗HNA-2a抗体在输血反应中的作用的表征方面已取得了多项进展。
NB1糖蛋白与内皮细胞黏附分子血小板内皮细胞黏附分子-1（PECAM-1）结合，并参与嗜中性粒细胞的迁移。嗜中性粒细胞CD177 mRNA的过表达已成为骨髓增殖性疾病（尤其是真性红细胞增多症）的一种有用但非特异性的生物标志物。CD177 mRNA过表达也是原发性血小板增多症患者中一部分血栓栓塞并发症风险增加的患者的生物标志物。在骨髓增殖性疾病患者中，CD177 mRNA过表达继发于JAK2（JAK2 V617F）的功能获得性突变。NB1糖蛋白与蛋白酶3共定位于嗜中性粒细胞质膜上，并且在韦格纳肉芽肿患者中，NB1糖蛋白和蛋白酶3的复合物可能通过抗中性粒细胞胞浆抗体引发嗜中性粒细胞的激活。血液成分中意外输注抗HNA-2a抗体经常会导致肺部输血反应。
CD177的表达是骨髓增殖性疾病的重要生物标志物，NB1糖蛋白是PECAM-1的配体，它可能在韦格纳肉芽肿中起作用，并且抗HNA-2a抗体经常导致肺部输血反应。
