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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
8
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pubmed:dateCreated |
2001-10-9
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pubmed:abstractText |
Cystic fibrosis (CF) is the most common, lethal autosomal recessive disease affecting children in the United States and Europe. Extensive work is being performed to develop both gene and drug therapies. The principal mutation causing CF is in the CFTR gene ([Delta F508]CFTR). This mutation causes the mutant protein to traffic poorly to the plasma membrane, and degrades CFTR chloride channel activity. CPX, a candidate drug for CF, binds to mutant CFTR and corrects the trafficking deficit. CPX also activates mutant CFTR chloride channel activity. CF airways are phenotypically inundated by inflammatory signals, primarily contributed by sustained secretion of the proinflammatory cytokine interleukin 8 (IL-8) from mutant CFTR airway epithelial cells. IL-8 production is controlled by genes from the TNF-alphaR/NFkappaB pathway, and it is possible that the CF phenotype is due to dysfunction of genes from this pathway. In addition, because drug therapy with CPX and gene therapy with CFTR have the same common endpoint of raising the levels of CFTR, we have hypothesized that either approach should have a common genomic endpoint.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
1076-1551
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
7
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
523-34
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pubmed:dateRevised |
2008-11-20
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pubmed:meshHeading |
pubmed-meshheading:11591888-Algorithms,
pubmed-meshheading:11591888-Cell Line,
pubmed-meshheading:11591888-Child,
pubmed-meshheading:11591888-Cluster Analysis,
pubmed-meshheading:11591888-Cystic Fibrosis,
pubmed-meshheading:11591888-Cystic Fibrosis Transmembrane Conductance Regulator,
pubmed-meshheading:11591888-Epithelial Cells,
pubmed-meshheading:11591888-Gene Expression Regulation,
pubmed-meshheading:11591888-Gene Therapy,
pubmed-meshheading:11591888-Humans,
pubmed-meshheading:11591888-Interleukin-8,
pubmed-meshheading:11591888-Lung,
pubmed-meshheading:11591888-Models, Biological,
pubmed-meshheading:11591888-NF-kappa B,
pubmed-meshheading:11591888-Oligonucleotide Array Sequence Analysis,
pubmed-meshheading:11591888-Pseudomonas aeruginosa,
pubmed-meshheading:11591888-Receptors, Tumor Necrosis Factor,
pubmed-meshheading:11591888-Respiratory Mucosa,
pubmed-meshheading:11591888-Time Factors,
pubmed-meshheading:11591888-Xanthines
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pubmed:year |
2001
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pubmed:articleTitle |
Control of the proinflammatory state in cystic fibrosis lung epithelial cells by genes from the TNF-alphaR/NFkappaB pathway.
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pubmed:affiliation |
Department of Anatomy, Institute of Molecular Medicine F. Edward Hebert School of Medicine, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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