Abstract

Cystic fibrosis (CF) is caused by a misfunctional CF transmembrane conductance regulator (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 ?F508 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 ?F508 CF patients with chronic rhinosinusitis (CRS) was pH = 7.08 ± 0.09 and was significantly lower compared to nasal epithelia from CRS patients 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)). The 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.

View details for DOI 10.1002/alr.20028

View details for Web of Science ID 000308912300009

View details for PubMedID 22034590

View details for PubMedCentralID PMC3199580