title: Ion and liquid transport across the bronchiolar epithelium.
abstract: The proper homeostasis of the airway surface liquid (ASL) depends on transepithelial ion and fluid transport and is critically important for lung defence, and more specifically for mucociliary transport. In cystic fibrosis (CF), abnormal ion and fluid transport lead to depleted ASL volume resulting in mucus plugs and recurrent lung infections. Like bronchi, human bronchioles exhibit amiloride-sensitive Na(+) absorption and cyclic-AMP and Ca(2+)-activated Cl(-) secretion. However, cyclic-AMP-stimulated Cl(-) and fluid secretion appears to be quantitatively more important in bronchioles than in bronchi. In CF bronchioles, like in CF bronchi, the ASL height is reduced because of an abnormally persistent Na(+) absorption, combined with a lacking CFTR-dependent Cl(-) secretion. The precocity and severity of the bronchiolar disease in CF could be attributed in part to the more important role of CFTR-dependent Cl(-) secretion and fluid secretion, and the lack of compensatory ATP-driven Cl(-) secretion and fluid secretion, in bronchioles compared to bronchi.
authors: Laboratoire de Biologie et Pharmacologie des Epithéliums Respiratoires, UPRES EA 220, Université de Versailles SQY, AP-HP, Hôpital Ambroise Paré, Service de Pneumologie, 9 Avenue Charles de Gaulle, 92104, Boulogne Cedex, France.
authors: Sabine
authors: S
authors: Blouquit-Laye
authors: Thierry
authors: T
authors: Chinet
mesh_terms: D000818:Animals; D001980:Bronchi / Q000166:cytology / Q000378:metabolism*; D002712:Chlorides / Q000378:metabolism*; D004848:Epithelium / Q000378:metabolism; D045604:Extracellular Fluid / Q000378:metabolism*; D006801:Humans; D017136:Ion Transport / Q000502:physiology; D020545:Respiratory Mucosa / Q000378:metabolism; D015398:Signal Transduction / Q000502:physiology; D012964:Sodium / Q000378:metabolism*; D014882:Water-Electrolyte Balance / Q000502:physiology*
chemical_list: D002712:Chlorides; D012964:Sodium