Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency
Cystic fibrosis, the most commonly inherited lethal pulmonary disorder in Caucasians, is
caused by mutations in the cystic fibrosis transmembrane conductance regulator gene …
caused by mutations in the cystic fibrosis transmembrane conductance regulator gene …
[HTML][HTML] CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes
The physiological components that contribute to cystic fibrosis (CF) lung disease are steadily
being elucidated. Gene therapy could potentially correct these defects. CFTR-null pigs …
being elucidated. Gene therapy could potentially correct these defects. CFTR-null pigs …
The evolution of cystic fibrosis care
JE Pittman, TW Ferkol - Chest, 2015 - Elsevier
Cystic fibrosis (CF) is the most common life-limiting inherited illness of whites. Most of the
morbidity and mortality in CF stems from impaired mucociliary clearance leading to chronic …
morbidity and mortality in CF stems from impaired mucociliary clearance leading to chronic …
Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model
PR Durie, G Kent, MJ Phillips, CA Ackerley - The American journal of …, 2004 - Elsevier
The lack of an appropriate animal model with multiorgan pathology characteristic of the
human form of cystic fibrosis has hampered our understanding of the pathobiology of the …
human form of cystic fibrosis has hampered our understanding of the pathobiology of the …
[HTML][HTML] Clinical implications of cystic fibrosis transmembrane conductance regulator mutations
JE Mickle, GR Cutting - Clinics in chest medicine, 1998 - Elsevier
Cystic fibrosis (CF) is a lethal autosomal recessive disorder characterized by obstructive
pulmonary disease, pancreatic exocrine deficiency, high content of sodium and chloride in …
pulmonary disease, pancreatic exocrine deficiency, high content of sodium and chloride in …
Cystic fibrosis transmembrane conductance regulator: roles in chronic obstructive pulmonary disease
M Dransfield, S Rowe, CF Vogelmeier… - American Journal of …, 2022 - atsjournals.org
Chronic obstructive pulmonary disease (COPD) manifests with a variety of clinical
presentations, reflecting its complex pathology. Currently, care focuses on symptom …
presentations, reflecting its complex pathology. Currently, care focuses on symptom …
CFTR dysfunction in cystic fibrosis and chronic obstructive pulmonary disease
E Fernandez Fernandez, C De Santi… - Expert review of …, 2018 - Taylor & Francis
Introduction: Obstructive lung diseases such as cystic fibrosis (CF) and chronic obstructive
pulmonary disease (COPD) are causes of high morbidity and mortality worldwide. CF is a …
pulmonary disease (COPD) are causes of high morbidity and mortality worldwide. CF is a …
Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/− Mice
EM Bruscia, PX Zhang, E Ferreira… - American journal of …, 2009 - atsjournals.org
Pulmonary infection with an exaggerated inflammatory response is the major cause of
morbidity and mortality in cystic fibrosis (CF). The objective of this study was to determine …
morbidity and mortality in cystic fibrosis (CF). The objective of this study was to determine …
Understanding how cystic fibrosis mutations disrupt CFTR function: from single molecules to animal models
Y Wang, JA Wrennall, Z Cai, H Li… - The international journal of …, 2014 - Elsevier
Defective epithelial ion transport is the hallmark of the life-limiting genetic disease cystic
fibrosis (CF). This abnormality is caused by mutations in the cystic fibrosis transmembrane …
fibrosis (CF). This abnormality is caused by mutations in the cystic fibrosis transmembrane …
Cystic fibrosis lung disease: an overview
NL Turcios - Respiratory care, 2020 - rc.rcjournal.com
Although better insights into the natural course of cystic fibrosis (CF) have led to treatment
approaches that have improved pulmonary health and increased the life expectancy of …
approaches that have improved pulmonary health and increased the life expectancy of …