NA+ TRANSPORT IN CYSTIC-FIBROSIS RESPIRATORY EPITHELIA - ABNORMAL BASAL RATE AND RESPONSE TO ADENYLATE-CYCLASE ACTIVATION
NA+ TRANSPORT IN CYSTIC-FIBROSIS RESPIRATORY EPITHELIA - ABNORMAL BASAL RATE AND RESPONSE TO ADENYLATE-CYCLASE ACTIVATION
复制标题
DOI：
10.1172/jci112708
复制
发表时间：
1986-11-01
影响因子：
15.9
通讯作者：
GATZY, JT
中科院分区：
文献类型：
--
作者：
BOUCHER, RC;STUTTS, MJ;GATZY, JT
囊性纤维化(CF)气道上皮细胞跨上皮电位差(PD)明显升高，氯离子通透性低。研究了活化的Na+吸收对PD的贡献，并试图增加CF上皮细胞的氯离子通透性。将来自CF和对照组的鼻黏膜上皮安装在Ussing小室中，并进行短路。与对照组相比，CF息肉对Na+的基础吸收率增加。β受体激动剂可诱导正常和特应性皮肤上皮细胞分泌Cl-，但β受体激动剂可进一步增加CF上皮细胞对Na+的吸收速率，但不包括Cl-的分泌。这种不寻常的效应不是由于异常的CFβ受体引起的，因为Forsklin也诱导了类似的效应，并且因为正常和CF上皮细胞产生的cAMP相似。我们认为，CF型呼吸道上皮细胞对Na+的吸收是加速的。对β激动剂的异常反应可能反映了cAMP调节通路的原发异常，或者反映了氯离子不通透的上皮细胞中cAMP调节的正常过程。
The transepithelial potential difference (PD) of cystic fibrosis (CF) airway epithleium is abormally raised and the Cl- permeability is low. E studied the contribution of active Na+ absorption to the PD and attempted to increase the Cl- permeability of CF epithelia. Nasal epithelia from CF and control subjects were mounted in Ussing chambers and were short-circuited. The basal rate of Na+ absorption was raised in CF polyps compared with control tissues. Whereas beta agonists induced Cl- secretion in normal and atopic epithelia, beta agonists further increased the rate of Na+ absorption in CF epithelia without including Cl- secretion. This unusual effect is not due to an abnormal CF beta receptor because similar effects were induced by forskolin, and because cAMP production was similar in normal and CF epithelia. We conclude that CF airway epithelia absorb Na+ at an accelerated rate. The abnormal response to beta agonists may reflect a primary abnormality in a cAMP-modulated path, or a normal cAMP-modulated process in a Cl- impermeable epithelial cell.