Impact of CFTR Modulation on Intestinal pH, Motility and Clinical Outcomes in Patients With Cystic Fibrosis and the G551D Mutation

Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor

(2015) Drucy Borowitz et al.   DIGESTIVE DISEASES AND SCIENCES

Equivalent substrates enable simultaneous study of gastrointestinal pH and CF-related diabetes

(2015) Drucy Borowitz et al.   Journal of Cystic Fibrosis

CFTR, bicarbonate, and the pathophysiology of cystic fibrosis

(2015) Drucy Borowitz   PEDIATRIC PULMONOLOGY

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

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

(2014) M. J. Hoegger et al.   SCIENCE

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

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

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

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Role of epithelial HCO3− transport in mucin secretion: lessons from cystic fibrosis

(2010) Paul M. Quinton   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

CFTR Functions as a Bicarbonate Channel in Pancreatic Duct Cells

(2009) Hiroshi Ishiguro et al.   JOURNAL OF GENERAL PHYSIOLOGY

Evidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review

(2008) Virginia A. Stallings et al.   JOURNAL OF THE AMERICAN DIETETIC ASSOCIATION

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

(2008) Paul M Quinton   LANCET