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 …
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 …
(CF). Although CF lung disease is primarily an infectious disorder, the associated …
Carbonic anhydrases in metazoan model organisms: molecules, mechanisms, and physiology
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 …
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 …
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 …
CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated …
Lung infections associated with cystic fibrosis
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 …
now recognized as a single disease whose diverse symptoms stem from the wide tissue …
Structure, gating, and regulation of the CFTR anion channel
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the ATP
binding cassette (ABC) transporter superfamily but functions as an anion channel crucial for …
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 …
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 …
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 …
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …