Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

Conditions Associated with the Cystic Fibrosis Defect Promote ChronicPseudomonas aeruginosaInfection

(2014) Benjamin J. Staudinger et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs

(2014) Mark J. Hoegger et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Differences in Disease Expression Between Primary Ciliary Dyskinesia and Cystic Fibrosis With and Without Pancreatic Insufficiency

(2013) Malena Cohen-Cymberknoh et al.   CHEST

The secret life of CFTR as a calcium-activated chloride channel

(2013) Arnaud Billet et al.   JOURNAL OF PHYSIOLOGY-LONDON

Why Mouse Airway Submucosal Gland Serous Cells Do Not Secrete Fluid in Response to cAMP Stimulation

(2012) Robert J. Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Produces Abnormalities in Tracheal Development in Neonatal Pigs and Young Children

(2010) David K. Meyerholz et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Quantification of Periciliary Fluid Height in Human Airway Biopsies Is Feasible, but Not Suitable as a Biomarker

(2010) Uta Griesenbach 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

Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs

(2010) Nam Soo Joo et al.   JOURNAL OF CLINICAL INVESTIGATION

Airway Mucus Function and Dysfunction

(2010) John V. Fahy et al.   NEW ENGLAND JOURNAL OF MEDICINE

Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth

(2010) D. A. Stoltz et al.   Science Translational Medicine

HCO3−Secretion by Murine Nasal Submucosal Gland Serous Acinar Cells during Ca2+-stimulated Fluid Secretion

(2008) Robert J. Lee et al.   JOURNAL OF GENERAL PHYSIOLOGY

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

(2008) Paul M Quinton   LANCET

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

(2008) C. S. Rogers et al.   SCIENCE