New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition

Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

(2019) Juliette Simonin et al.   Scientific Reports

The mucin bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation

(2018) Anna Ermund et al.   EUROPEAN RESPIRATORY JOURNAL

The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

(2018) Patrick J. Moore et al.   EXPERT OPINION ON THERAPEUTIC TARGETS

Granule-stored MUC5B mucins are packed by the non-covalent formation of N-terminal head-to-head tetramers

(2018) Sergio Trillo-Muyo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte

(2018) Lindsey W. Plasschaert et al.   NATURE

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

(2018) Daniel T. Montoro et al.   NATURE

Meconium ileus in Cystic Fibrosis

(2017) Meghana Sathe et al.   Journal of Cystic Fibrosis

Linking increased airway hydration, ciliary beating, and mucociliary clearance through ENaC inhibition

(2015) Annika B. M. Åstrand et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Hyper-osmolarity and calcium chelation: Effects on cystic fibrosis mucus

(2015) Anna Ermund et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

Genome-wide association meta-analysis identifies five modifier loci of lung disease severity in cystic fibrosis

(2015) Harriet Corvol et al.   Nature Communications

Hypertonic saline releases the attached small intestinal cystic fibrosis mucus

(2014) Anna Ermund et al.   CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY

Lung disease modifier genes in cystic fibrosis

(2014) Loic Guillot et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Microbial-induced meprin   cleavage in MUC2 mucin and a functional CFTR channel are required to release anchored small intestinal mucus

(2014) A. Schutte et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Studies of mucus in mouse stomach, small intestine, and colon. I. Gastrointestinal mucus layers have different properties depending on location as well as over the Peyer's patches

(2013) Anna Ermund et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Molecular transport machinery involved in orchestrating luminal acid-induced duodenal bicarbonate secretionin vivo

(2013) Anurag Kumar Singh et al.   JOURNAL OF PHYSIOLOGY-LONDON

Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype

(2012) Jenny K. Gustafsson et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis

(2012) Lei Sun et al.   NATURE GENETICS

Calcium and pH-dependent packing and release of the gel-forming MUC2 mucin

(2012) D. Ambort et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An ex vivo method for studying mucus formation, properties, and thickness in human colonic biopsies and mouse small and large intestinal explants

(2011) Jenny K. Gustafsson et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients

(2011) Carla Colombo et al.   Journal of Cystic Fibrosis

Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis

(2011) N. Klymiuk et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Pharmacological Properties of N-(3,5-Diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine Methanesulfonate (552-02), a Novel Epithelial Sodium Channel Blocker with Potential Clinical Efficacy for Cystic Fibrosis Lung Disease

(2008) A. J. Hirsh et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS