Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
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Pass the bicarb: the importance of HCO3– for mucin releaseUpdate in Cystic Fibrosis 2014Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing miceRescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770Molecular mechanism of pancreatic and salivary gland fluid and HCO3 secretionThe Evolution of Cystic Fibrosis CareReal-time imaging of exocytotic mucin release and swelling in Calu-3 cells using acridine orange.Loss of cftr function leads to pancreatic destruction in larval zebrafish.Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airwaysCystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectivesOrigins of cystic fibrosis lung diseaseLoss of carbonic anhydrase XII function in individuals with elevated sweat chloride concentration and pulmonary airway diseaseFunctionalized positive nanoparticles reduce mucin swelling and dispersionMicrobial, host and xenobiotic diversity in the cystic fibrosis sputum metabolomeSubstance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process.Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype.Microstructural alterations of sputum in cystic fibrosis lung disease.Marked increases in mucociliary clearance produced by synergistic secretory agonists or inhibition of the epithelial sodium channel.Lineage-specific expression of bestrophin-2 and bestrophin-4 in human intestinal epithelial cellsImpact of CFTR Modulation on Intestinal pH, Motility, and Clinical Outcomes in Patients With Cystic Fibrosis and the G551D Mutation.Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice.Native small airways secrete bicarbonate.The switch of intestinal Slc26 exchangers from anion absorptive to HCOFormula secretory mode is dependent on CFTR anion channel function.Mimicking the host and its microenvironment in vitro for studying mucosal infections by Pseudomonas aeruginosa.Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping resultsMicrobial-induced meprin β cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus.A new role for bicarbonate in mucus formation.VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.A functional anatomic defect of the cystic fibrosis airway.Conditions associated with the cystic fibrosis defect promote chronic Pseudomonas aeruginosa infectionPhysiological relevance of cell-specific distribution patterns of CFTR, NKCC1, NBCe1, and NHE3 along the crypt-villus axis in the intestine.Embryonic frog epidermis: a model for the study of cell-cell interactions in the development of mucociliary disease.CFTR and lung homeostasis.Hypertonic saline releases the attached small intestinal cystic fibrosis mucusThe ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs.Biogeochemical forces shape the composition and physiology of polymicrobial communities in the cystic fibrosis lung.Serine proteases degrade airway mucins in cystic fibrosis.Defective adenosine-stimulated cAMP production in cystic fibrosis airway epithelia: a novel role for CFTR in cell signaling.Impaired mucosal barrier function in the small intestine of the cystic fibrosis mouse.Redox biology of the intestine
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P2860
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on August 2008
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@en
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@nl
type
label
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@en
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@nl
prefLabel
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@en
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@nl
P1433
P1476
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis.
@en
P2093
Paul M Quinton
P304
P356
10.1016/S0140-6736(08)61162-9
P407
P577
2008-08-01T00:00:00Z