publication . Article . Other literature type . 2008

17β-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia

Coakley, Ray D.; Sun, Hengrui; Clunes, Lucy A.; Rasmussen, Julia E.; Stackhouse, James R.; Okada, Seiko F.; Fricks, Ingrid; Young, Steven L.; Tarran, Robert;
Open Access
  • Published: 20 Nov 2008 Journal: Journal of Clinical Investigation (issn: 0021-9738, Copyright policy)
  • Publisher: American Society for Clinical Investigation
Abstract
Normal airways homeostatically regulate the volume of airway surface liquid (ASL) through both cAMP- and Ca2+-dependent regulation of ion and water transport. In cystic fibrosis (CF), a genetic defect causes a lack of cAMP-regulated CFTR activity, leading to diminished Cl– and water secretion from airway epithelial cells and subsequent mucus plugging, which serves as the focus for infections. Females with CF exhibit reduced survival compared with males with CF, although the mechanisms underlying this sex-related disadvantage are unknown. Despite the lack of CFTR, CF airways retain a limited capability to regulate ASL volume, as breathing-induced ATP release acti...
Subjects
Medical Subject Headings: respiratory system
free text keywords: General Medicine, Intracellular, Purinergic receptor, Estrogen, medicine.drug_class, medicine, Cystic fibrosis, medicine.disease, Water transport, Secretion, Internal medicine, medicine.medical_specialty, business.industry, business, Homeostasis, Cystic fibrosis transmembrane conductance regulator, biology.protein, biology, Endocrinology, Research Article
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