Department for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
Biomedical Research in Endstage and Obstructive Lung Disease, German Center for Lung Research, Hannover, Germany.
J Clin Invest. 2023 Oct 16;133(20):e174667. doi: 10.1172/JCI174667.
In the lungs, the cystic fibrosis transmembrane conductance regulator (CFTR) regulates ion transport in surface-airway epithelia and submucosal glands, thus determining airway surface liquid (ASL) volume and mucus hydration. In this issue of the JCI, Lei Lei and colleagues report that the CFTR-rich and barttin/Cl- channel-expressing ionocytes mediate chloride absorption across airway epithelia, whereas the more abundant basal cells and secretory cells mediate chloride secretion. Thus, CFTR-mediated secretion and absorption of chloride ions in the lung are segregated by cell type, which has implications for future molecular therapies for cystic fibrosis lung disease.
在肺部,囊性纤维化跨膜电导调节因子(CFTR)调节气道表面上皮和黏膜下腺中的离子转运,从而决定气道表面液体(ASL)的体积和黏液水合作用。在本期 JCI 中,雷磊及其同事报告称,富含 CFTR 和 barttin/Cl-通道的离子细胞介导气道上皮的氯离子吸收,而更为丰富的基底细胞和分泌细胞则介导氯离子分泌。因此,肺部 CFTR 介导的氯离子分泌和吸收是由细胞类型分隔的,这对囊性纤维化肺病的未来分子治疗具有重要意义。