Mattes P M, Maloney P C, Littlefield J W
Proc Natl Acad Sci U S A. 1987 May;84(9):3009-13. doi: 10.1073/pnas.84.9.3009.
An abnormal regulation of chloride permeability has been described for epithelial cells from patients with cystic fibrosis (CF). To learn more about the biochemical basis of this inherited disease, we have studied chloride metabolism in cultured CF fibroblasts by comparing the efflux of 36Cl- from matched pairs of CF and normal fibroblasts. The rate constants describing 36Cl- efflux did not differ between the two cell types, but in each of the four pairs tested the amount of 36Cl- contained within CF cells was consistently reduced, by 25-30%, relative to normal cells. Comparisons of cell water content and 22Na+ efflux showed no differences between the two cell types, suggesting that overall intracellular chloride concentration is lower than normal in CF fibroblasts. Such data suggest that the CF gene defect is expressed in skin fibroblasts and that this defect may alter the regulation of intracellular Cl- concentration, perhaps through changes in Cl- permeability.
囊性纤维化（CF）患者上皮细胞的氯离子通透性存在异常调节。为了更多地了解这种遗传性疾病的生化基础，我们通过比较来自配对的CF和正常成纤维细胞的³⁶Cl⁻流出情况，研究了培养的CF成纤维细胞中的氯代谢。描述³⁶Cl⁻流出的速率常数在两种细胞类型之间没有差异，但在测试的四对细胞中，相对于正常细胞，CF细胞内所含的³⁶Cl⁻量始终减少了25%-30%。细胞含水量和²²Na⁺流出的比较显示两种细胞类型之间没有差异，这表明CF成纤维细胞中细胞内总体氯离子浓度低于正常水平。这些数据表明CF基因缺陷在皮肤成纤维细胞中表达，并且这种缺陷可能通过改变Cl⁻通透性来改变细胞内Cl⁻浓度的调节。
