Pulmonary Neuroendocrine Cells, Airway Innervation, and Smooth Muscle Are Altered in Cftr Null Mice

• DOI: 10.1165/rcmb.2005-0468oc
• Publication Date: 2006-04-14T01:25:27Z
• Authors: Jie Pan; Catherine Luk; Geraldine Kent; Ernest Cutz; Herman Yeger
• Keywords: Synaptophysin

The amine- and peptide-producing pulmonary neuroendocrine cells (PNEC) are widely distributed within the airway mucosa of mammalian lung as solitary innervated clusters, neuroepithelial bodies (NEB), which function O2 sensors. These express Cftr hence could play a role in pathophysiology cystic fibrosis (CF) disease. We performed confocal microscopy morphometric analysis on sections from Cftr-/- (null), Cftr+/+, Cftr+/- (control) mice at developmental stages E20, P5, P9, P30 to determine distribution, frequency, innervation PNEC/NEB, cell mass smooth muscle, neuromuscular junctions using synaptic vesicle protein 2, muscle actin, synaptophysin markers, respectively. mean number PNEC/NEB was significantly reduced compared with control whereas comparable or increased numbers were observed postnatally. NEB null showed significant reduction intracorpuscular nerve endings mice, is consistent an intrinsic abnormality PNEC system. airways density (approximately 20-30%) innervation, decreased 35%), 20%) mice. conclude that exhibit heretofore unappreciated structural alterations affecting cellular neural components system its resulting blunted sensing tonus. development system, muscle.