Okano M, Sakiyama Y, Matsumoto S, Mizuno F, Osato T
Department of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan.
J Clin Lab Anal. 1995;9(1):77-9. doi: 10.1002/jcla.1860090115.
Previously, we have reported significantly lower immunoglobulin (Ig) A production in supernatants of cultured lymphoblastoid cells using enzyme-linked immunosorbent assay from patients with ataxia-telangiectasia (AT) when compared to that of age- and sex-matched healthy individuals. Here, we further assess the degree of cytoplasmic Ig production in these cells and also analyze it during the early phase of Epstein-Barr virus immortalization. All classes of cytoplasmic IgM, IgG, and IgA productions were demonstrated in cells from healthy controls. In contrast, cells from patients with AT showed only cytoplasmic IgM and IgG with low or nondetectable levels of IgA during and after the immortalizing process. These results suggest B lymphocytes bearing IgA are functionally immature and/or defective in patients with AT.
此前，我们曾报道，与年龄和性别匹配的健康个体相比，采用酶联免疫吸附测定法检测发现，共济失调毛细血管扩张症（AT）患者培养的淋巴母细胞上清液中的免疫球蛋白（Ig）A产量显著降低。在此，我们进一步评估这些细胞中细胞质Ig的产生程度，并在爱泼斯坦-巴尔病毒永生化的早期阶段对其进行分析。健康对照者的细胞中均显示出所有类型的细胞质IgM、IgG和IgA产物。相比之下，AT患者的细胞在永生化过程中和之后仅显示出细胞质IgM和IgG，而IgA水平较低或无法检测到。这些结果表明，携带IgA的B淋巴细胞在AT患者中功能不成熟和/或存在缺陷。
