Cl- secretion by trachea of CFTR (+/-) and (-/-) fetal mouse.
Cl- secretion by trachea of CFTR (+/-) and (-/-) fetal mouse.
复制标题
CFTR (/-) 和 (-/-) 胎儿小鼠气管分泌的 Cl-。
DOI：
10.1165/ajrcmb.13.3.7544595
复制
发表时间：
1995
期刊：
影响因子：
--
通讯作者：
Gatzy,JT
中科院分区：
文献类型：
--
作者：
Barker,PM;Brigman,KK;Paradiso,AM;Boucher,RC;Gatzy,JT
新生儿囊性纤维化 (CF) 肺没有病理变化表明胎儿 CF 肺充满正常体积的液体，并且 Cl- 通过囊性纤维化跨膜电导调节器 (CFTR) 相关 Cl- 通道以外的路径分泌。我们研究了与 CFTR (-/-)“敲除”雄性交配的 16 至 19 天妊娠 CFTR (+/-)（杂合）雌性胎儿小鼠的远端肺液体含量和跨上皮电位差 (PD)。远端肺水含量不受胎儿基因型的影响。基础PD没有差异（CFTR（+/-），8.6 mV和CFTR（-/-），9.1 mV），并且两组的PD均通过腔内注射阿米洛利（10（-4）M）（-25％）和在浴液中添加布美他尼（10（-4）M）（-40％）后受到抑制。特布他林 (3 x 10(-5) M) 在两组中均引起类似的 PD 增加（约 65%）。腔内注射离子霉素（2 x 10(-5) 和 5 x 10(-6) M）均使两组的 PD 升高（CFTR (+/-) 分别增加 32% 和 27%，CFTR (-/-) 分别增加 41% 和 11%）。布美他尼抑制所有由特布他林和离子霉素诱导的 PD 增加。用离子霉素或Ca2+螯合剂BAPTA (10(-4) M)预处理不会减弱特布他林的PD反应。离子霉素或 ATP，但不是特布他林，可以增加分离培养的气管上皮细胞的细胞内 Ca2+ 浓度。（摘要截断为 250 字）
The absence of pathologic changes in newborn cystic fibrosis (CF) lung suggests that the fetal CF lung is inflated with a normal volume of liquid and that Cl- is secreted through paths other than the cystic fibrosis transmembrane conductance regulator (CFTR)-associated Cl- channel. We studied liquid content of distal lung and transepithelial electrical potential difference (PD) of cultured cystic tracheal explants from 16 to 19 day gestation fetal mice of CFTR (+/-)(heterozygous) females that were mated with CFTR (-/-) "knockout" males. Distal lung water content was not affected by fetal genotype. Basal PDs were not different (CFTR (+/-), 8.6 mV, and CFTR (-/-), 9.1 mV), and PDs of both groups were inhibited by intraluminal injection of amiloride (10(-4) M) (-25%) and after addition of bumetanide (10(-4) M) to the bath (-40%). Terbutaline (3 x 10(-5) M) induced a similar increase in PD (about 65%) in both groups. Intraluminal injection of ionomycin (2 x 10(-5) and 5 x 10(-6) M) raised PD in both groups (CFTR (+/-) by 32 and 27% and CFTR (-/-) by 41 and 11%). All of the increase in PD induced by terbutaline and ionomycin was inhibited by bumetanide. The PD response to terbutaline was not attenuated by pretreatment with ionomycin or the Ca2+ chelator BAPTA (10(-4) M). Ionomycin or ATP, but not terbutaline, increased intracellular Ca2+ concentration of isolated cultured tracheal epithelial cells.(ABSTRACT TRUNCATED AT 250 WORDS)