Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride ion channel, but its relationship to the primary clinical manifestation of CF, chronic Pseudomonas aeruginosa pulmonary infection, is unclear.
|
9342367 |
1997 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) is the main cause of fetal lung infection in cystic fibrosis patients.
|
10437775 |
1999 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
CFTR mutations and host susceptibility to Pseudomonas aeruginosa lung infection.
|
11834374 |
2002 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
However, the molecular mechanism by which the deltaF508-CFTR protein facilitates pulmonary infection and inflammation remains unclear.
|
11934265 |
2002 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
A central question is how defective CFTR function in CF leads to chronic lung infection and deterioration of lung function.
|
12475759 |
2003 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
These data suggest that mutations in the CFTR in men with CBAVD, in addition to causing infertility, lead to subclinical bacterial pulmonary infection and inflammation consistent with mild CF.
|
14551163 |
2004 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Resistance to Pseudomonas aeruginosa chronic lung infection requires cystic fibrosis transmembrane conductance regulator-modulated interleukin-1 (IL-1) release and signaling through the IL-1 receptor.
|
17283089 |
2007 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy.
|
17443603 |
2007 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Microbial lung infections are the major cause of morbidity and mortality in the hereditary metabolic disorder cystic fibrosis, yet the molecular mechanisms leading from the mutation of cystic fibrosis transmembrane conductance regulator (CFTR) to lung infection are still unclear.
|
18376404 |
2008 |
Infectious Lung Disorder
|
0.100 |
AlteredExpression
|
group |
BEFREE |
We conclude that an intrinsic alteration of function is linked to the absence of CFTR from skeletal muscle, leading to dysregulated calcium homeostasis, augmented inflammatory/atrophic gene expression signatures, and increased diaphragmatic weakness during pulmonary infection.
|
19649303 |
2009 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Neonatal CFTR-knockout ferrets demonstrated many of the characteristics of human CF disease, including defective airway chloride transport and submucosal gland fluid secretion; variably penetrant meconium ileus (MI); pancreatic, liver, and vas deferens disease; and a predisposition to lung infection in the early postnatal period.
|
20739752 |
2010 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Understanding the multitude of effects of CFTR on mucosal physiology and susceptibility and progression of chronic lung disease, and how host immune responses fail to adequately control lung infection, will be essential for the development of improved therapies for CF.
|
22753926 |
2012 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy.
|
23076917 |
2012 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Cystic fibrosis (CF) is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, and is characterized by chronic pulmonary infections.
|
23617438 |
2013 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy.
|
24282073 |
2013 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel gene results in dehydration, mucus plugging, and reduction of the airway surface liquid layer (ASL) height which favour chronic lung infection and neutrophil based inflammation leading to progressive lung destruction and early death of people with CF.
|
25866809 |
2015 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Cystic fibrosis is caused by a defective gene encoding a protein called the cystic fibrosis transmembrane conductance regulator (CFTR), and is characterised by chronic lung infection resulting in inflammation and progressive lung damage that results in a reduced life expectancy.
|
27314455 |
2016 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage.
|
27709245 |
2017 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
The arrival of new cystic fibrosis transmembrane conductance-regulator (CFTR)-correcting therapies will bring more opportunities to prevent the disease, apart from only treating chronic lung infection.
|
27913761 |
2017 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Importantly, WT BMC delivery delayed Pseudomonas aeruginosa lung infection and increased survival of CFTR<sup>-/-</sup> recipients.
|
28187947 |
2017 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Recent progress in the development of drugs to correct CFTR deficiency thus may be decisive in the control of P. aeruginosa lung infection.
|
30620748 |
2019 |
Infectious Lung Disorder
|
0.100 |
GeneticVariation
|
group |
BEFREE |
Cystic fibrosis (CF) is an inherited disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene that promotes persistent lung infection and inflammation and progressive loss of lung function.
|
31262295 |
2019 |
Infectious Lung Disorder
|
0.100 |
Biomarker
|
group |
BEFREE |
Moreover, massive evidences show that defective CFTR gives rise to extracellular GSH level decrease and elevated glucose concentrations in airway surface liquid (ASL), thus encouraging lung infection by pathogens in the CF advancement.
|
31698802 |
2019 |