Origins of cystic fibrosis lung disease

DA Stoltz, DK Meyerholz, MJ Welsh - New England Journal of …, 2015 - Mass Medical Soc
Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

[HTML][HTML] Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

AM Cantin, D Hartl, MW Konstan, JF Chmiel - Journal of Cystic Fibrosis, 2015 - Elsevier
Lung disease is the major cause of morbidity and mortality in patients with cystic fibrosis
(CF). Although CF lung disease is primarily an infectious disorder, the associated …

Carbonic anhydrases in metazoan model organisms: molecules, mechanisms, and physiology

A Aspatwar, MEE Tolvanen, H Barker… - Physiological …, 2022 - journals.physiology.org
During the past three decades, mice, zebrafish, fruit flies, and Caenorhabditis elegans have
been the primary model organisms used for the study of various biological phenomena …

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

AA Pezzulo, XX Tang, MJ Hoegger, MH Abou Alaiwa… - Nature, 2012 - nature.com
Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

F Van Goor, S Hadida… - Proceedings of the …, 2009 - National Acad Sciences
Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the
CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated …

Lung infections associated with cystic fibrosis

JB Lyczak, CL Cannon, GB Pier - Clinical microbiology reviews, 2002 - Am Soc Microbiol
While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is
now recognized as a single disease whose diverse symptoms stem from the wide tissue …

Structure, gating, and regulation of the CFTR anion channel

L Csanády, P Vergani, DC Gadsby - Physiological reviews, 2019 - journals.physiology.org
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …

High-fat diet modifies the PPAR-γ pathway leading to disruption of microbial and physiological ecosystem in murine small intestine

J Tomas, C Mulet, A Saffarian… - Proceedings of the …, 2016 - National Acad Sciences
Diet is among the most important factors contributing to intestinal homeostasis, and basic
functions performed by the small intestine need to be tightly preserved to maintain health …

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation

JP Clancy, SM Rowe, FJ Accurso, ML Aitken, RS Amin… - Thorax, 2012 - thorax.bmj.com
Background VX-809, a cystic fibrosis transmembrane conductance regulator (CFTR)
modulator, has been shown to increase the cell surface density of functional F508del-CFTR …

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

MJ Hoegger, AJ Fischer, JD McMenimen… - Science, 2014 - science.org
Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …