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Acid and base secretion in freshly excised nasal tissue from cystic fibrosis patients with ΔF508 mutation
Author(s) -
Cho DoYeon,
Hwang Peter H.,
Illek Beate,
Fischer Horst
Publication year - 2011
Publication title -
international forum of allergy and rhinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.503
H-Index - 46
eISSN - 2042-6984
pISSN - 2042-6976
DOI - 10.1002/alr.20028
Subject(s) - cystic fibrosis , cystic fibrosis transmembrane conductance regulator , secretion , medicine , δf508 , nasal polyps , submucosal glands , mucous membrane of nose , ussing chamber , mucus , forskolin , endocrinology , pathology , biology , receptor , ecology
Background 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 ΔF508 mutation. Methods 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. Results The equilibrium pH of nasal epithelia from ΔF508 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 HCO 3 − secretory rate was further increased by forskolin by 16.1% in normal, but not in CF tissues. Conclusion 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. © 2011 ARS‐AAOA, LLC.