Ion composition and rheology of airway liquid from cystic fibrosis fetal tracheal xenografts
Ion composition and rheology of airway liquid from cystic fibrosis fetal tracheal xenografts
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DOI：
10.1165/ajrcmb.20.4.3264
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发表时间：
1999-04-01
影响因子：
6.4
通讯作者：
Puchelle, E
中科院分区：
文献类型：
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作者：
Baconnais, S;Tirouvanziam, R;Puchelle, E
气道表面液体（ASL）的组成部分取决于活性离子和水通过呼吸上皮的运输。通常认为，在囊性纤维化（CF）中，CF跨膜电导调节蛋白异常导致ASL的离子组成失衡和脱水，导致异常的流变学和转运特性。为了探讨离子组成，水含量和气道液体（AL）的粘度之间的关系，我们使用了严重联合免疫缺陷（SCID）小鼠的胎儿气道的人异种移植模型。将胎儿气管的6个非CF和6个CF部分皮下移植到在无病原体条件下饲养的SCID小鼠的侧腹。植入后9 ~ 17周，收集聚集在封闭的圆柱形移植物中的AL。在AL取样时，所有气管移植物均显示分化良好的假复层表面上皮和粘膜下腺体。使用受控应力流变仪测量AL的粘度。用X射线微区分析法对铝的离子组成进行了定量分析。在非CF之间没有观察到AL粘度的显著差异（0.6 +/- 0.5 Pa . s）和CF（0.2 +/- 0.1 Pa . s）样品。在来自非CF和CF样品的AL中，离子浓度为Na：63.9 +/- 7.6，79.7 +/- 11.6; Cl：64.9 +/- 13.2，82.6 +/- 15.7; Mg：1.9 +/- 0.3，2.2 +/- 0.4; S：4.9 +/- 1.3，4.8 +/- 0.5; K：2.4 +/- 0.5，3.2 +/- 1.6;和Ca：1.2 +/- 0.3，2.6 +/- 0.8 mmol/L。来自CF与非CF异种移植物的AL的离子组成没有显著差异。这些结果表明，在炎症和感染之前，胎儿AL的粘度和离子组成在CF和非CF中没有差异。
The composition of airway surface liquid (ASL) is partly determined by active ion and water transport through the respiratory epithelium. It is usually stated that in cystic fibrosis (CF), CF transmembrane conductance regulator protein abnormality results in imbalanced ion composition and dehydration of ASL, leading to abnormal theologic and transport properties. To explore the relationship between ion composition, water content, and viscosity of airway liquid (AL), we used a human xenograft model of fetal airways developed in severe combined immunodeficiency (SCID) mice. Six non-CF and six CF portions of fetal tracheas were engrafted subcutaneously in the flanks of SCID mice raised in pathogen-free conditions. AL accumulated in the closed cylindric grafts was harvested 9 to 17 wk after implantation. At the time of AL sampling, all tracheal grafts displayed well-differentiated pseudostratified surface epithelium and submucosal glands. The viscosity of AL was measured using a controlled-stress rheometer. The ion composition of AL was quantified by X-ray microanalysis. No significant difference was observed for AL viscosity between non-CF (0.6 +/- 0.5 Pa . s) and CF (0.2 +/- 0.1 Pa . s) samples. In AL from non-CF and CF samples, the ion concentrations were Na: 63.9 +/- 7.6, 79.7 +/- 11.6; Cl: 64.9 +/- 13.2, 82.6 +/- 15.7; Mg: 1.9 +/- 0.3, 2.2 +/- 0.4; S: 4.9 +/- 1.3, 4.8 +/- 0.5; K: 2.4 +/- 0.5, 3.2 +/- 1.6; and Ca: 1.2 +/- 0.3, 2.6 +/- 0.8 mmol/liter, respectively. The ion composition of AL from CF versus non-CF xenografts was not significantly different. These results suggest that prior to inflammation and infection, the viscosity and ion composition of the fetal AL do not differ in CF and non-CF.