[HTML][HTML] 'CFTR-opathies': disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations

PG Noone, MR Knowles - Respiratory research, 2001 - Springer
Cystic fibrosis is a genetic disease that is associated with abnormal sweat electrolytes, sino-
pulmonary disease, exocrine pancreatic insufficiency, and male infertility. Insights into …

Extracellular nucleotide signaling along the renal epithelium

EM Schwiebert, BK Kishore - American Journal of …, 2001 - journals.physiology.org
During the past two decades, several cell membrane receptors, which preferentially bind
extracellular nucleotides, and their analogs have been identified. These receptors …

The relative roles of passive surface forces and active ion transport in the modulation of airway surface liquid volume and composition

R Tarran, BR Grubb, JT Gatzy, CW Davis… - The Journal of general …, 2001 - rupress.org
Two hypotheses have been proposed recently that offer different views on the role of airway
surface liquid (ASL) in lung defense. The “compositional” hypothesis predicts that ASL …

[HTML][HTML] The CF salt controversy: in vivo observations and therapeutic approaches

R Tarran, BR Grubb, D Parsons, M Picher, AJ Hirsh… - Molecular cell, 2001 - cell.com
There is controversy over whether abnormalities in the salt concentration or volume of
airway surface liquid (ASL) initiate cystic fibrosis (CF) airway disease. In vivo studies of CF …

[HTML][HTML] Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH

S Jayaraman, Y Song, L Vetrivel… - The Journal of …, 2001 - Am Soc Clin Investig
The concentration of salt in the thin layer of fluid at the surface of large airways, the airway-
surface liquid (ASL), is believed to be of central importance in airway physiology and in the …

Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na+] and pH but elevated viscosity

S Jayaraman, NS Joo, B Reitz… - Proceedings of the …, 2001 - National Acad Sciences
Fluid and macromolecule secretion by submucosal glands in mammalian airways is
believed to be important in normal airway physiology and in the pathophysiology of cystic …

[HTML][HTML] Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell …

GM Braunstein, RM Roman, JP Clancy… - Journal of Biological …, 2001 - ASBMB
These studies provide evidence that cystic fibrosis transmembrane conductance regulator
(CFTR) potentiates and accelerates regulatory volume decrease (RVD) following hypotonic …

[HTML][HTML] Cystic fibrosis pathogens activate Ca2+-dependent mitogen-activated protein kinase signaling pathways in airway epithelial cells

AJ Ratner, R Bryan, A Weber, S Nguyen… - Journal of Biological …, 2001 - ASBMB
Much of the pulmonary disease in cystic fibrosis is associated with polymorphonuclear
leukocyte-dominated airway inflammation caused by bacterial infection. Respiratory …

Lactoferrin binds CpG-containing oligonucleotides and inhibits their immunostimulatory effects on human B cells

BE Britigan, TS Lewis, M Waldschmidt… - The Journal of …, 2001 - journals.aai.org
Unmethylated CpG dinucleotide motifs in bacterial DNA, as well as oligodeoxynucleotides
(ODN) containing these motifs, are potent stimuli for many host immunological responses …

Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract

TH Schroeder, N Reiniger, G Meluleni… - The Journal of …, 2001 - journals.aai.org
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) has been proposed
to be an epithelial cell receptor for Pseudomonas aeruginosa involved in bacterial …