Ueda E, Nishimura J, Kitani T, Nasu K, Kageyama T, Kim Y U, Takeda J, Kinoshita T
Department of Internal Medicine, Osaka University, Japan.
Int Immunol. 1992 Nov;4(11):1263-71. doi: 10.1093/intimm/4.11.1263.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired type hemolytic disorder. Hematopoietic cells of patients with PNH are deficient in glycosylphosphatidylinositol (GPI) anchored membrane proteins. Since some membrane-bound complement inhibitors, such as CD59 and decay accelerating factor (DAF), are GPI anchored proteins, abnormal cells from patients with PNH are sensitive to complement attack. Their myeloid and erythroid cells are affected more than their lymphoid cells. Patients whose B cells were severely deficient in GPI anchored proteins were chosen to establish cell lines by Epstein-Barr virus mediated transformation. The lines established (SS-1-, TK-1-, and TK-14- cell lines) had the following characteristics of PNH. First, GPI anchored proteins were completely absent from the surface of SS-1- and TK-14- cells, and were expressed at very low levels on TK-1- cells, whereas polypeptide anchored proteins were normally expressed on these three lines. Secondly, DAF mRNAs of the SS-1- cell line were qualitatively and quantitatively indistinguishable from those of a control, wild-type cell line. Third, pro-CD59 and pro-DAF molecules were detected intracellularly in these cell lines, their pro-CD59 being smaller and more hydrophilic than that from a wild-type cell line. These cell lines should be useful in further studies on the pathogenesis of PNH.
阵发性睡眠性血红蛋白尿（PNH）是一种获得性溶血性疾病。PNH患者的造血细胞缺乏糖基磷脂酰肌醇（GPI）锚定膜蛋白。由于一些膜结合补体抑制剂，如CD59和衰变加速因子（DAF），是GPI锚定蛋白，PNH患者的异常细胞对补体攻击敏感。他们的髓系和红系细胞比淋巴细胞受到的影响更大。选择B细胞严重缺乏GPI锚定蛋白的患者，通过爱泼斯坦-巴尔病毒介导的转化建立细胞系。所建立的细胞系（SS-1-、TK-1-和TK-14-细胞系）具有以下PNH的特征。首先，SS-1-和TK-14-细胞表面完全没有GPI锚定蛋白，而在TK-1-细胞上表达水平极低，而多肽锚定蛋白在这三个细胞系上正常表达。其次，SS-1-细胞系的DAF mRNA在质量和数量上与对照野生型细胞系的DAF mRNA没有区别。第三，在这些细胞系的细胞内检测到前体CD59和前体DAF分子，它们的前体CD59比野生型细胞系的前体CD59更小且更具亲水性。这些细胞系将有助于进一步研究PNH的发病机制。
