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

Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways

(2013) Alessandra Livraghi-Butrico et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs

(2013) David A. Stoltz et al.   JOURNAL OF CLINICAL INVESTIGATION

ENaC Inhibitors and Airway Re-hydration in Cystic Fibrosis: State of the Art

(2013) Mike Althaus   Current Molecular Pharmacology

ENaCs and ASICs as therapeutic targets

(2012) Yawar J. Qadri et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Effect of nitric oxide on epithelial ion transports in noncystic fibrosis and cystic fibrosis human proximal and distal airways

(2012) Sabine Blouquit-Laye et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

The CFTR and ENaC debate: how important is ENaC in CF lung disease?

(2012) James F. Collawn et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

A mutation in the β-subunit of ENaC identified in a patient with cystic fibrosis-like symptoms has a gain-of-function effect

(2012) Robert Rauh et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

AMP-activated protein kinase (AMPK)-dependent and -independent pathways regulate hypoxic inhibition of transepithelial Na+ transport across human airway epithelial cells

(2012) CD Tan et al.   BRITISH JOURNAL OF PHARMACOLOGY

Proteases, cystic fibrosis and the epithelial sodium channel (ENaC)

(2012) P. H. Thibodeau et al.   CELL AND TISSUE RESEARCH

Organization of the ENaC-regulatory machinery

(2012) Rama Soundararajan et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Epithelial Na+ channel regulation by cytoplasmic and extracellular factors

(2012) Ossama B. Kashlan et al.   EXPERIMENTAL CELL RESEARCH

Activation of the Epithelial Sodium Channel (ENaC) by the Alkaline Protease fromPseudomonas aeruginosa

(2012) Michael B. Butterworth et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular organization of the mucins and glycocalyx underlying mucus transport over mucosal surfaces of the airways

(2012) M Kesimer et al.   Mucosal Immunology

CFTR Regulates Early Pathogenesis of Chronic Obstructive Lung Disease in βENaC-Overexpressing Mice

(2012) Bjarki Johannesson et al.   PLoS One

Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease

(2011) B. R. Grubb et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Distribution of human PLUNC/BPI fold-containing (BPIF) proteins

(2011) Lynne Bingle et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Regulation and dysregulation of epithelial Na+ channels

(2011) Lawrence G. Palmer et al.   Clinical and Experimental Nephrology

The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease

(2011) Zhe Zhou et al.   Journal of Cystic Fibrosis

Cleavage of endogenous γENaC and elevated abundance of αENaC are associated with increased Na+ transport in response to apical fluid volume expansion in human H441 airway epithelial cells

(2011) Chong D. Tan et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease

(2011) T. Kimura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Acute Regulation of the Epithelial Sodium Channel in Airway Epithelia by Proteases and Trafficking

(2010) Michael M. Myerburg et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Loss of Anion Transport without Increased Sodium Absorption Characterizes Newborn Porcine Cystic Fibrosis Airway Epithelia

(2010) Jeng-Haur Chen et al.   CELL

The Cystic Fibrosis Transmembrane Conductance Regulator Impedes Proteolytic Stimulation of the Epithelial Na+Channel

(2010) Martina Gentzsch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human Alveolar Type II Cells Secrete and Absorb Liquid in Response to Local Nucleotide Signaling

(2010) Peter F. Bove et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Airway Surface Liquid Volume Regulation Determines Different Airway Phenotypes in Liddle Compared with βENaC-overexpressing Mice

(2010) Marcus A. Mall et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+self inhibition

(2010) Robert Rauh et al.   JOURNAL OF PHYSIOLOGY-LONDON

Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases

(2010) Erol A. Gaillard et al.   PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY

The Multifunctional Host Defense Peptide SPLUNC1 Is Critical for Homeostasis of the Mammalian Upper Airway

(2010) Glen McGillivary et al.   PLoS One

Characterization of the Epithelial Sodium Channel δ-Subunit in Human Nasal Epithelium

(2009) Nadine Bangel-Ruland et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage

(2009) A. Garcia-Caballero et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Tracheobronchial air-liquid interface cell culture: a model for innate mucosal defense of the upper airways?

(2008) Mehmet Kesimer et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Genetic Analysis of Rwandan Patients With Cystic Fibrosis-Like Symptoms

(2008) Léon Mutesa et al.   CHEST

Role of the amiloride-sensitive epithelial Na+channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease

(2008) Marcus A. Mall   EXPERIMENTAL PHYSIOLOGY

Insight toward epithelial Na+channel mechanism revealed by the acid-sensing ion channel 1 structure

(2008) James D. Stockand et al.   IUBMB LIFE

Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs

(2008) C. S. Rogers et al.   SCIENCE