Kemp T, Schram-Doumont A, van Geffel R, Kram R, Szpirer C
Pediatr Res. 1986 Jun;20(6):520-6. doi: 10.1203/00006450-198606000-00008.
In order to determine whether cystic fibrosis neutrophils are affected in their secretory functions, lysosomal enzyme release and chemiluminescence (light emission from cells) were assayed in patients' cells and compared with those in normal control cells. We observed a decreased response of cystic fibrosis neutrophils in beta-glucuronidase release and chemiluminescence after stimulation by N-formyl-methionyl-leucyl-phenylalanine. There was no significant correlation of these results with the clinical score nor with the medical treatment. On the other hand, responses to the calcium ionophore A23187 and to opsonized zymosan showed no significant difference between normal and cystic fibrosis subjects in lysosomal enzyme release. N-formyl-methionyl-leucyl-phenylalanine receptor alterations did not seem involved in the observed effect as demonstrated by Scatchard plot analysis of N-formyl-methionyl-leucyl-phenylalanine binding to these receptors. These results clearly demonstrate a difference between normal and cystic fibrosis neutrophils in release and chemiluminescence responses to N-formyl-methionyl-leucyl-phenylalanine stimulation, a difference that might be located in the plasma membrane as both responses are membrane dependent.
为了确定囊性纤维化患者的中性粒细胞分泌功能是否受到影响，对患者细胞中的溶酶体酶释放和化学发光（细胞发光）进行了检测，并与正常对照细胞进行比较。我们观察到，在受到N-甲酰甲硫氨酰-亮氨酰-苯丙氨酸刺激后，囊性纤维化患者中性粒细胞的β-葡萄糖醛酸酶释放和化学发光反应有所降低。这些结果与临床评分或治疗方法均无显著相关性。另一方面，在溶酶体酶释放方面，正常人和囊性纤维化患者对钙离子载体A23187和调理酵母聚糖的反应没有显著差异。通过对N-甲酰甲硫氨酰-亮氨酰-苯丙氨酸与这些受体结合的Scatchard图分析表明，N-甲酰甲硫氨酰-亮氨酰-苯丙氨酸受体改变似乎与观察到的效应无关。这些结果清楚地表明，正常中性粒细胞和囊性纤维化中性粒细胞在对N-甲酰甲硫氨酰-亮氨酰-苯丙氨酸刺激的释放和化学发光反应方面存在差异，这种差异可能位于质膜，因为这两种反应都依赖于膜。
