CFTR, bicarbonate, and the pathophysiology of cystic fibrosis

Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis

(2014) Steven M. Rowe et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

A Functional Anatomic Defect of the Cystic Fibrosis Airway

(2014) Susan E. Birket et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Pseudomonas aeruginosa in Cystic Fibrosis Patients With G551D-CFTR Treated With Ivacaftor

(2014) Sonya L. Heltshe et al.   CLINICAL INFECTIOUS DISEASES

Neonates with cystic fibrosis have a reduced nasal liquid pH; A small pilot study

(2014) Mahmoud H. Abou Alaiwa et al.   Journal of Cystic Fibrosis

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37

(2014) Mahmoud H. Abou Alaiwa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Mechanisms of CFTR Functional Variants That Impair Regulated Bicarbonate Permeation and Increase Risk for Pancreatitis but Not for Cystic Fibrosis

(2014) Jessica LaRusch et al.   PLoS Genetics

The buffer capacity of airway epithelial secretions

(2014) Dusik Kim et al.   Frontiers in Physiology

Short Palate, Lung, and Nasal Epithelial Clone–1 Is a Tightly Regulated Airway Sensor in Innate and Adaptive Immunity

(2013) Clemente J. Britto et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Native Small Airways Secrete Bicarbonate

(2013) A. K. M. Shamsuddin et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Escherichia coli Dysbiosis Correlates With Gastrointestinal Dysfunction in Children With Cystic Fibrosis

(2013) Lucas R. Hoffman et al.   CLINICAL INFECTIOUS DISEASES

Unraveling the complex genetic model for cystic fibrosis: pleiotropic effects of modifier genes on early cystic fibrosis-related morbidities

(2013) Weili Li et al.   HUMAN GENETICS

Normal mucus formation requires cAMP-dependent HCO3−secretion and Ca2+-mediated mucin exocytosis

(2013) Ning Yang et al.   JOURNAL OF PHYSIOLOGY-LONDON

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

(2013) A. L. Garland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) Mike Althaus   Current Molecular Pharmacology

Deletion of Slc26a6 alters the stoichiometry of apical Cl−/HCO3− exchange in mouse pancreatic duct

(2012) Ying Song et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Mucociliary transport in porcine trachea: differential effects of inhibiting chloride and bicarbonate secretion

(2012) Jeffrey L. Cooper et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule

(2012) Daniel Gelfond et al.   DIGESTIVE DISEASES AND SCIENCES

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

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

Cross-Sectional and Longitudinal Comparisons of the Predominant Fecal Microbiota Compositions of a Group of Pediatric Patients with Cystic Fibrosis and Their Healthy Siblings

(2011) Gwen Duytschaever et al.   APPLIED AND ENVIRONMENTAL MICROBIOLOGY

Dynamic Regulation of CFTR Bicarbonate Permeability by [Cl−]i and Its Role in Pancreatic Bicarbonate Secretion

(2010) Hyun Woo Park et al.   GASTROENTEROLOGY

Pancreatic Bicarbonate Secretion Involves Two Proton Pumps

(2010) Ivana Novak et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator–dependent bicarbonate secretion

(2009) Mary Abigail S. Garcia et al.   JOURNAL OF CLINICAL INVESTIGATION

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

(2008) Paul M Quinton   LANCET