Boucher R C, Ross D W, Knowles M R, Gatzy J T, Parker J C
Pediatr Res. 1984 Dec;18(12):1336-9. doi: 10.1203/00006450-198412000-00024.
Recent studies have identified abnormalities in Cl- permeation across two target cystic fibrosis (CF) epithelia (sweat duct and respiratory epithelium). In the present study, anion conductances of red blood cells (RBCs) and peripheral blood lymphocytes (PBLs) from CF and normal subjects were estimated and compared. For RBCs, the valinomycin-induced rate constant for K+ loss (PK+) was taken as an index of PCl-. For PBLs, the secondary volume increase after gramicidin pretreatment and hypotonic (0.67 X isotonic) stress was used to estimate PCl-. The Cl- permeabilities of RBCs and PBLs from CF and control subjects were comparable. These findings suggest that the abnormality in PCl- reported for CF sweat ductal and respiratory epithelia is not expressed in circulating blood elements.
最近的研究已经确定了氯离子跨两个目标囊性纤维化（CF）上皮细胞（汗腺导管和呼吸道上皮细胞）渗透的异常情况。在本研究中，对来自CF患者和正常受试者的红细胞（RBC）和外周血淋巴细胞（PBL）的阴离子电导进行了估计和比较。对于红细胞，将缬氨霉素诱导的钾离子流失速率常数（PK+）作为氯离子通透性（PCl-）的指标。对于外周血淋巴细胞，短杆菌肽预处理和低渗（0.67倍等渗）应激后的二次体积增加用于估计氯离子通透性。CF患者和对照受试者的红细胞和外周血淋巴细胞的氯离子通透性相当。这些发现表明，CF汗腺导管和呼吸道上皮细胞中报道的氯离子通透性异常在循环血液成分中未表现出来。
