Conod E J, Conover J H, Hirschhorn K
Pediatr Res. 1975 Sep;9(9):724-9. doi: 10.1203/00006450-197509000-00008.
The ability of epsilon-amino caproic acid (EACA)-treated normal serum and of cystic fibrosis (CF)-affected and carrier sera to promote the release of lysosomal enzymes from sensitized human polymorphonuclear leukocytes (PMN) was assessed through the measurement of beta-glucuronidase and myeloperoxidase activity after exposure of these cells to the various test sera. This study was initiated to extend the analogies between preciliary dyskinesia factor (pre-CDF), separated from the cell-free media of cultures derived from CF homozygous and heterozygous individuals, and C3a anaphylatoxin. The extent of lysosomal degranulation of human PMN exposed to fresh untreated sera of each of five controls, seven CF homozygotes, and eight heterozygotes, as expressed by the amount of beta-glucuronidase releases, was 7.84% (+/- 0.934) for countrol sera, 14.01% (+/- 1.79) for CF-affected sera, and 10.61% (+/- 1.43) for heterozygous sera. The difference between CF homozygotes and control subjects is significatn (P less than 0.0001), as is the difference between CF-affected and carrier individuals (0.001 less than P less than 0.005) and between control subjects and carriers (0.001 less than P less than 0.005), when beta-glucuronidase. However, the differences between control subjects and CF heterozygous individuals are not significant. Treatment of these sera with 1 M EACA gave values for beta-glucuronidase and myeloperoxidase release which are slightly reduced when compared with those obtained with fresh, untreated samples. EACA apparently reduces the activity of beta-glucuronidase released from PMN. Amicon filtration studies of these serum samples demonstrated that degranulating ability and the presence of cilicary dyskinesia, as assessed by rabbit tracheal bioassay, are not always associated. Therefore, the relationship between pre-CDF and the degranulator activity in native CF-affected and carrier sera is unclear, in part because of the limitations inherent in the test systems employed.
通过测量经各种测试血清处理后的人多形核白细胞（PMN）中β-葡萄糖醛酸酶和髓过氧化物酶的活性，评估了用ε-氨基己酸（EACA）处理的正常血清、囊性纤维化（CF）患者血清和携带者血清促进致敏人多形核白细胞释放溶酶体酶的能力。开展这项研究是为了拓展在从不明原因的纤毛运动障碍因子（pre-CDF，从CF纯合子和杂合子个体来源的无细胞培养基中分离得到）和C3a过敏毒素之间的相似性。用五个对照组、七个CF纯合子和八个杂合子的新鲜未处理血清处理人PMN后，溶酶体脱颗粒的程度（以β-葡萄糖醛酸酶释放量表示），对照组血清为7.84%（±0.934），CF患者血清为14.01%（±1.79），杂合子血清为10.61%（±1.43）。当以β-葡萄糖醛酸酶衡量时，CF纯合子与对照受试者之间的差异具有显著性（P小于0.0001），CF患者与携带者个体之间的差异也具有显著性（0.001小于P小于0.005），对照受试者与携带者之间的差异同样具有显著性（0.001小于P小于0.005）。然而，对照受试者与CF杂合子个体之间的差异不显著。用1M EACA处理这些血清后，β-葡萄糖醛酸酶和髓过氧化物酶的释放值与新鲜未处理样本相比略有降低。EACA显然降低了PMN释放的β-葡萄糖醛酸酶的活性。对这些血清样本进行的Amicon过滤研究表明，通过兔气管生物测定评估的脱颗粒能力和纤毛运动障碍的存在并不总是相关的。因此，pre-CDF与天然CF患者和携带者血清中的脱颗粒活性之间的关系尚不清楚，部分原因是所采用的测试系统存在固有局限性。
