Kissel Karin, Scheffler Steffi, Kerowgan Mohammed, Bux Jürgen
Institute for Clinical Immunology and Transfusion Medicine, Giessen, Germany.
Blood. 2002 Jun 1;99(11):4231-3. doi: 10.1182/blood.v99.11.4231.
Alloimmunization to the neutrophil antigen NB1 (HNA-2a, CD177) can result in immune neutropenia and transfusion-related acute lung injury. Recently, we were able to elucidate the primary structure of NB1. To shed light also on the molecular basis of the NB1-negative phenotype, we studied the neutrophils of 2 women with NB1-specific alloantibodies for intracellular and extracellular NB1 expression, NB1-specific mRNA production, and the presence of the NB1 gene. No antibody binding to neutrophils was observed by immunofluorescence and immunoblot using a variety of human and monoclonal NB1-specific antibodies. By reverse transcription-polymerase chain reaction with NB1-specific primers we could not detect NB1 cDNAs without accessory sequences, which were found to be introns. The NB1 gene was present in the genome of both patients. Our data indicate that the NB1-negative phenotype is the result of different off-frame insertions on RNA level, resulting in NB1 deficiency on neutrophils.
对中性粒细胞抗原NB1（HNA-2a，CD177）的同种免疫可导致免疫性中性粒细胞减少和输血相关急性肺损伤。最近，我们得以阐明NB1的一级结构。为了也阐明NB1阴性表型的分子基础，我们研究了2名患有NB1特异性同种抗体的女性的中性粒细胞，以检测细胞内和细胞外NB1表达、NB1特异性mRNA产生以及NB1基因的存在情况。使用多种人和单克隆NB1特异性抗体，通过免疫荧光和免疫印迹未观察到抗体与中性粒细胞结合。通过使用NB1特异性引物的逆转录-聚合酶链反应，我们无法检测到没有辅助序列的NB1 cDNA，这些辅助序列被发现是内含子。两名患者的基因组中均存在NB1基因。我们的数据表明，NB1阴性表型是RNA水平上不同移码插入的结果，导致中性粒细胞上NB1缺乏。
