囊性纤维化患者经异丙肾上腺素刺激的成纤维细胞中3':5'-环磷酸腺苷水平异常。
Abnormal levels of 3':5'-cyclic AMP in isoproterenol-stimulated fibroblasts from patients with cystic fibrosis.
作者信息
Buchwald M
出版信息
Proc Natl Acad Sci U S A. 1976 Aug;73(8):2899-903. doi: 10.1073/pnas.73.8.2899.
To determine if the abnormalities of exocrine secretion characteristic of cystic fibrosis could be investigated in vitro, I studied the synthesis of 3':5'-cyclic AMP after isoproterenol stimulation in skin fibroblasts derived from patients with cystic fibrosis and from normal individuals. Comparison of normal and cystic fibrosis cells showed that the latter had 2- to 5-fold greater levels of intracellular cyclic AMP after stimulation with isoproterenol. The difference between the strains was observed at every stage of the culture cycle and was specific to stimulation by beta-adrenergic agonists. It could not be accounted for by different dose- or time-response curves nor by leakage of cyclic AMP into the medium. The increased sensitivity to catecholamines may reflect an intrinsic genetic property of cystic fibrosis cells, and it may be feasible to use this system to study the biochemical basis of the genetic defect in cystic fibrosis.
为了确定能否在体外研究囊性纤维化所特有的外分泌分泌异常情况，我研究了来自囊性纤维化患者和正常个体的皮肤成纤维细胞在异丙肾上腺素刺激后3':5'-环磷酸腺苷（3':5'-cyclic AMP）的合成情况。正常细胞与囊性纤维化细胞的比较显示，在用异丙肾上腺素刺激后，后者细胞内的环磷酸腺苷水平高出2至5倍。在培养周期的每个阶段都观察到了这两种细胞系之间的差异，且这种差异特定于β-肾上腺素能激动剂的刺激。它不能用不同的剂量-反应曲线或时间-反应曲线来解释，也不能用环磷酸腺苷泄漏到培养基中来解释。对儿茶酚胺敏感性的增加可能反映了囊性纤维化细胞的一种内在遗传特性，利用这个系统来研究囊性纤维化遗传缺陷的生化基础可能是可行的。
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