Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency

Y Xu, JC Clark, BJ Aronow, CR Dey, C Liu… - Journal of Biological …, 2003 - ASBMB
Cystic fibrosis, the most commonly inherited lethal pulmonary disorder in Caucasians, is
caused by mutations in the cystic fibrosis transmembrane conductance regulator gene …

[HTML][HTML] CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes

B Steines, DD Dickey, J Bergen, KJDA Excoffon… - JCI insight, 2016 - ncbi.nlm.nih.gov
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 …

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 …

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 …

[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 …

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 …

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 …

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 …

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 …

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 …