Acid and base secretion in freshly excised nasal tissue from cystic fibrosis patients with ΔF508 mutation.
Acid and base secretion in freshly excised nasal tissue from cystic fibrosis patients with ΔF508 mutation.
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DOI：
10.1002/alr.20028
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发表时间：
2011-03
影响因子：
6.4
通讯作者：
Fischer, Horst
中科院分区：
文献类型：
--
作者：
Cho, Do-Yeon;Hwang, Peter H.;Illek, Beate;Fischer, Horst
关键词：
囊性纤维化（CF）是由功能失调的CFTR蛋白引起的，CFTR蛋白被认为有助于调节气道表面液体（ASL）pH。本研究调查了Δ F508突变纯合子CF患者新鲜切除的人鼻组织中的酸碱分泌。在鼻窦手术过程中收集人鼻粘膜，并在Ussing室中进行研究。粘液平衡pH值和速率的酸和碱分泌使用pH统计技术测定。慢性鼻窦炎（CRS）患者鼻上皮细胞的平衡pH值为7.08 ± 0.09，显著低于无CF CRS患者鼻上皮细胞的平衡pH值（7.33 ± 0.06）和正常人鼻上皮细胞的平衡pH值（7.34 ± 0.08，n = 6）。CF鼻组织的碱分泌速率为11.8 ± 2.4 nmol·min-1·cm-2，显著低于正常值（57.2 ± 9.2 nmol·min-1·cm-2）。在正常组织中，毛喉素使HCO3 −分泌率进一步增加16.1%，但在CF组织中没有增加。我们的数据表明，CF患者表现出显着较低的鼻气道上皮细胞的碱分泌。这是可能的，在CF ASL pH值的调节不当可能会产生负面影响的先天宿主防御系统。
Cystic fibrosis (CF) is caused by a misfunctional CFTR protein, which is believed to contributes to the regulation of the airway surface liquid (ASL) pH. This study investigated acid and base secretion in freshly excised human nasal tissues from CF patients homozygous for the ΔF508 mutation. Human nasal mucosa was collected during sinus surgery and investigated in Ussing chambers. Mucosal equilibrium pH values and rate of acid and base secretion were determined using the pH-stat technique. The equilibrium pH of nasal epithelia from ΔF508 CF patients with chronic rhinosinusitis (CRS) was pH = 7.08 ± 0.09 and significantly lower compared to nasal epithelia from CRS without CF (pH = 7.33 ± 0.06) and normal subjects (pH = 7.34 ± 0.08, n = 6). The rate of base secretion in CF nasal tissues was 11.8 ± 2.4 nmol·min−1·cm−2, which was significantly lower than normal (57.2 ± 9.2 nmol·min−1·cm−2). HCO3−secretory rate was further increased by forskolin by 16.1% in normal, but not in CF tissues. Our data suggests that CF patients exhibited significantly lower base secretion by the nasal airway epithelium. It is possible that improper regulation of ASL pH in CF may negatively impact the innate host defense system.
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DOI：
10.1165/ajrcmb.26.1.4425
发表时间：
2002-01-01
影响因子：
6.4
作者：
Nakayama, K;Jia, YX;Sasaki, H
通讯作者：
Sasaki, H
DOI：
10.1152/nips.01412.2002
发表时间：
2003-02-01
期刊：
NEWS IN PHYSIOLOGICAL SCIENCES
影响因子：
--
作者：
Hug, MJ;Tamada, T;Bridges, RJ
通讯作者：
Bridges, RJ
影响因子：
3
作者：
CORKILL, JE;SISSON, PR;HART, CA
通讯作者：
HART, CA
影响因子：
2.6
作者：
Cho DY;Hajighasemi M;Hwang PH;Illek B;Fischer H
通讯作者：
Fischer H
影响因子：
5.5
作者：
Song, YL;Salinas, D;Verkman, AS
通讯作者：
Verkman, AS