Hyposecretion, Not Hyperabsorption, Is the Basic Defect of Cystic Fibrosis Airway Glands*♦

NS Joo, T Irokawa, RC Robbins, JJ Wine - Journal of Biological Chemistry, 2006 - ASBMB
Human airways and glands express the anion channel cystic fibrosis transmembrane
conductance regulator, CFTR, and the epithelial Na+ channel, ENaC. Cystic fibrosis (CF) …

Effect of anion transport inhibition on mucus secretion by airway submucosal glands

SK Inglis, MR Corboz, AE Taylor… - American Journal of …, 1997 - journals.physiology.org
To model the airway glandular defect in cystic fibrosis (CF), the effect of anion secretion
blockers on submucosal gland mucus secretion was investigated. Porcine distal bronchi …

Functional interaction of CFTR and ENaC in sweat glands

M Reddy, P Quinton - Pflügers Archiv, 2003 - Springer
The cystic fibrosis transmembrane conductance regulator (CFTR) plays a significant role in
transepithelial salt absorption as well as secretion by a number of epithelial tissues …

Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands

NS Joo, T Irokawa, JV Wu, RC Robbins… - Journal of Biological …, 2002 - ASBMB
We are testing the hypothesis that the malfunctioning of airway gland serous cells is a
component of cystic fibrosis (CF) airway disease. CF is caused by mutations that disrupt CF …

Acinar origin of CFTR-dependent airway submucosal gland fluid secretion

JV Wu, ME Krouse, JJ Wine - American Journal of …, 2007 - journals.physiology.org
Cystic fibrosis (CF) airway disease arises from defective innate defenses, especially
defective mucus clearance of microorganisms. Airway submucosal glands secrete most …

Why mouse airway submucosal gland serous cells do not secrete fluid in response to cAMP stimulation

RJ Lee, JK Foskett - Journal of Biological Chemistry, 2012 - ASBMB
Airway submucosal glands are important sites of cystic fibrosis transmembrane conductance
regulator (CFTR) chloride (Cl−) channel expression and fluid secretion in the airway …

Submucosal gland dysfunction as a primary defect in cystic fibrosis

D Salinas, PM Haggie, JR Thiagarajah… - The FASEB …, 2005 - Wiley Online Library
It has been proposed that defective submucosal gland function in CF airways is a major
determinant of CF airway disease. We tested the hypothesis that submucosal gland function …

Fluid transport across cultures of human tracheal glands is altered in cystic fibrosis

C Jiang, WE Finkbeiner, JH Widdicombe… - The Journal of …, 1997 - Wiley Online Library
1 There is evidence that defective submucosal gland secretion contributes to the airway
pathology of cystic fibrosis (CF). Using a capacitance probe technique, we have compared …

Does epithelial sodium channel hyperactivity contribute to cystic fibrosis lung disease?

CA Hobbs, C Da Tan, R Tarran - The Journal of physiology, 2013 - Wiley Online Library
Key points• Lung hydration and mucus clearance rates are set by a balance between CFTR‐
mediated Cl− secretion and ENaC‐led Na+ absorption. In CF airways, CFTR is diminished …

Cftr and ENaC ion channels mediate NaCl absorption in the mouse submandibular gland

MA Catalán, T Nakamoto… - The Journal of …, 2010 - Wiley Online Library
Cystic fibrosis is caused by mutations in CFTR, the cystic fibrosis transmembrane
conductance regulator gene. Disruption of CFTR‐mediated anion conductance results in …