Dimanche M T, Le Deist F, Fischer A, Arnaout M A, Griscelli C, Lisowska-Grospierre B
Eur J Immunol. 1987 Mar;17(3):417-9. doi: 10.1002/eji.1830170318.
The defective membrane expression of the adhesive protein family (LFA-1, Mo1 and p150,93) on leukocytes from certain patients with recurrent bacterial infections was shown to be secondary to the absence of synthesis of mature beta chain that is common to all three antigens (Springer et al., 1984, Lisowska-Grospierre et al., 1986). In all patients, studies of beta-chain biosynthesis that lead to this conclusion were performed using the monoclonal anti-beta chain antibody to isolate the beta subunit. Since this antibody detects the mature form of beta chain only, the potential presence of a precursor or of an abnormal beta chain in the patient's cells could not be tested. The availability of the polyclonal antibody to the purified beta subunit allowed us to re-examine the biosynthesis of the LFA-1 subunits in 3 affected children. In all 3 patients, the absence of membrane expression of the LFA-1, CR3 and p150,95 proteins was confirmed. The LFA-1 alpha-chain precursor of 170 kDa was detected in the lysates of PHA blasts of two children, but was not detected in the third. The beta-chain precursor of 85 kDa was isolated by the polyclonal anti-beta chain antiserum from the cytoplasm of phytohemagglutinin and Epstein-Barr virus-induced blasts of one patient. The same antibody precipitated some peptides of smaller mol. wt. from the cell lysates of 2 other patients. These results suggest that in this disorder the membrane nonexpression of the adhesive proteins is probably due to the structural abnormality of beta chain which, although synthesized, is rapidly degradated.
某些复发性细菌感染患者白细胞上黏附蛋白家族（淋巴细胞功能相关抗原-1、Mac-1和p150,93）的膜表达缺陷被证明是由于缺乏这三种抗原共有的成熟β链合成所致（施普林格等人，1984年；利索夫斯卡-格罗斯皮埃尔等人，1986年）。在所有患者中，使用单克隆抗β链抗体分离β亚基进行了导致这一结论的β链生物合成研究。由于该抗体仅检测β链的成熟形式，因此无法检测患者细胞中前体或异常β链的潜在存在。针对纯化β亚基的多克隆抗体的可用性使我们能够重新检查3名患病儿童中淋巴细胞功能相关抗原-1亚基的生物合成。在所有3名患者中，均证实了淋巴细胞功能相关抗原-1、补体受体3和p150,95蛋白的膜表达缺失。在两名儿童的PHA刺激淋巴细胞裂解物中检测到了170 kDa的淋巴细胞功能相关抗原-1α链前体，但在第三名儿童中未检测到。通过多克隆抗β链抗血清从一名患者的植物血凝素和爱泼斯坦-巴尔病毒诱导的淋巴细胞胞质中分离出了85 kDa的β链前体。同一抗体从另外两名患者的细胞裂解物中沉淀出了一些分子量较小的肽段。这些结果表明，在这种疾病中，黏附蛋白的膜无表达可能是由于β链的结构异常，尽管β链已合成，但会迅速降解。
