Department of Pharmacology and Physiology, University of Rochester Medical Center, Rochester, NY 14620, USA.
J Physiol. 2010 Feb 15;588(Pt 4):713-24. doi: 10.1113/jphysiol.2009.183541. Epub 2009 Dec 21.
Cystic fibrosis is caused by mutations in CFTR, the cystic fibrosis transmembrane conductance regulator gene. Disruption of CFTR-mediated anion conductance results in defective fluid and electrolyte movement in the epithelial cells of organs such as the pancreas, airways and sweat glands, but the function of CFTR in salivary glands is unclear. Salivary gland acinar cells produce an isotonic, plasma-like fluid, which is subsequently modified by the ducts to produce a hypotonic, NaCl-depleted final saliva. In the present study we investigated whether submandibular salivary glands (SMGs) in F508 mice (Cftr(F/F)) display ion transport defects characteristic of cystic fibrosis in other tissues. Immunolocalization and whole-cell recordings demonstrated that Cftr and the epithelial Na(+) (ENaC) channels are co-expressed in the apical membrane of submandibular duct cells, consistent with the significantly higher saliva [NaCl] observed in vivo in Cftr(F/F) mice. In contrast, Cftr and ENaC channels were not detected in acinar cells, nor was saliva production affected in Cftr(F/F) mice, implying that Cftr contributes little to the fluid secretion process in the mouse SMG. To identify the source of the NaCl absorption defect in Cftr(F/F) mice, saliva was collected from ex vivo perfused SMGs. Cftr(F/F) glands secreted saliva with significantly increased [NaCl]. Moreover, pharmacological inhibition of either Cftr or ENaC in the ex vivo SMGs mimicked the Cftr(F/F) phenotype. In summary, our results demonstrate that NaCl absorption requires and is likely to be mediated by functionally dependent Cftr and ENaC channels localized to the apical membranes of mouse salivary gland duct cells.
囊性纤维化是由 CFTR 基因突变引起的,CFTR 是囊性纤维化跨膜电导调节基因。CFTR 介导的阴离子电导的破坏导致胰腺、气道和汗腺等器官的上皮细胞中液体和电解质的运动缺陷,但 CFTR 在唾液腺中的功能尚不清楚。唾液腺腺泡细胞产生等渗、血浆样液体,随后被导管修饰,产生低渗、NaCl 耗尽的最终唾液。在本研究中,我们研究了 F508 小鼠(Cftr(F/F)) 的下颌下腺 (SMG) 是否显示出与其他组织中囊性纤维化特征一致的离子转运缺陷。免疫定位和全细胞记录表明,Cftr 和上皮 Na+(ENaC) 通道在颌下腺导管细胞的顶膜上共同表达,这与 Cftr(F/F) 小鼠体内观察到的显著更高的唾液[NaCl]一致。相比之下,在腺泡细胞中未检测到 Cftr 和 ENaC 通道,Cftr(F/F) 小鼠的唾液产生也不受影响,这意味着 Cftr 对小鼠 SMG 的液体分泌过程贡献很小。为了确定 Cftr(F/F) 小鼠中 NaCl 吸收缺陷的来源,从离体灌流的 SMG 中收集唾液。Cftr(F/F) 腺体分泌的唾液[NaCl]显著增加。此外,在离体 SMG 中药理学抑制 Cftr 或 ENaC 模拟了 Cftr(F/F) 表型。总之,我们的结果表明,NaCl 吸收需要并可能由定位于小鼠唾液腺导管细胞顶膜的功能性依赖 Cftr 和 ENaC 通道介导。