[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 …
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 …
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 …
surface liquid (ASL) in lung defense. The “compositional” hypothesis predicts that ASL …
[HTML][HTML] The CF salt controversy: in vivo observations and therapeutic approaches
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 …
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 …
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 …
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 …
(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 …
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 …
(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 …
to be an epithelial cell receptor for Pseudomonas aeruginosa involved in bacterial …