A. Weber et al., CHLORIDE CONDUCTANCE IN NASAL FIBROBLASTS OF PATIENTS WITH AND WITHOUT CYSTIC-FIBROSIS - RELEVANCE FOR THE OTOLARYNGOLOGIST, HNO. Hals-, Nasen-, Ohrenarzte, 45(9), 1997, pp. 695-701
Cystic fibrosis (CF) is a complex systemic disease that has pathologic
al alterations in the upper airways, including the recurrent formation
of nasal polyps. Although the fibroblast is the predominant cell type
in nasal stroma and nasal polyps, little is known about the electroph
ysiological properties of nasal fibroblasts. We investigated whether f
ibroblasts possess a cAMP-regulated chloride conductance which is impa
ired in patients with CF. Thus far the few studies concerning conducta
nce in fibroblasts have been performed on skin fibroblasts using indir
ect methods and have yielded conflicting results. Therefore we studied
chloride conductance in fused nasal fibroblasts by employing conventi
onal microelectrodes. We have demonstrated that a cAMP-regulated chlor
ide conductance is present in fibroblasts. However, this chloride cond
uctance cannot be activated in fibroblasts from CF-patients. Thus, we
present direct evidence that the impairment of the cAMP-regulated chlo
ride conductance in CF is not confined to epithelial cells but also af
fects the fibroblast. We discuss how this conductance might modulate f
ibroblast proliferation to produce polyp formation.