Physiological Institute, University of Regensburg, University Street 31, 93053, Germany.
Physiological Institute, University of Regensburg, University Street 31, 93053, Germany.
J Cyst Fibros. 2017 Nov;16(6):653-662. doi: 10.1016/j.jcf.2017.06.005. Epub 2017 Jul 18.
Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene encoding CF transmembrane conductance regulator (CFTR), which is a chloride and bicarbonate channel necessary for fluid secretion and extracellular alkalization. For a long time, research concentrated on abnormal Cl and Na transport, but neglected bicarbonate as a crucial factor in CF.
The present short review reports early findings as well as recent insights into the role of CFTR for bicarbonate transport and its defects in CF.
The available data indicate impaired bicarbonate transport not only in pancreas, intestine, airways, and reproductive organs, but also in salivary glands, sweat duct and renal tubular epithelial cells. Defective bicarbonate transport is closely related to the impaired mucus properties and mucus blocking in secretory organs of CF patients, causing the life threatening lung disease.
Apart from the devastating lung disease, abrogated bicarbonate transport also leads to many other organ dysfunctions, which are outlined in the present review.
囊性纤维化（CF，粘液化脓性疾病）是由编码 CF 跨膜电导调节因子（CFTR）的基因突变引起的，CFTR 是一种氯离子和碳酸氢根离子通道，对于液体分泌和细胞外碱化是必需的。长期以来，研究集中在异常的 Cl 和 Na 转运上，但忽视了碳酸氢根作为 CF 的一个关键因素。
本简短综述报告了 CFTR 对碳酸氢盐转运及其在 CF 中的缺陷的早期发现以及最近的研究进展。
现有数据表明，不仅在胰腺、肠道、气道和生殖器官，而且在唾液腺、汗腺导管和肾管状上皮细胞中，碳酸氢盐转运都受到损害。碳酸氢盐转运缺陷与 CF 患者分泌器官中黏液特性和黏液阻塞受损密切相关，导致危及生命的肺部疾病。
除了破坏性的肺部疾病外，碳酸氢盐转运的中断还导致许多其他器官功能障碍，本综述概述了这些器官功能障碍。
