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Status |
Public on Mar 24, 2006 |
Title |
Transcriptional adaptation to cystic fibrosis transmembrane conductance regulator deficiency |
Organism |
Mus musculus |
Experiment type |
Expression profiling by array
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Summary |
Cystic fibrosis, the most commonly inherited lethal pulmonary disorder in Caucasians, is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). To identify genomic responses to the presence or absence of CFTR in pulmonary tissues in vivo, microarray analyses of lung mRNAs were performed on whole lung tissue from mice lacking (CFTR(-)) or expressing mouse CFTR (CFTR(+)). Whereas the histology of lungs from CFTR(-) and CFTR(+) mice was indistinguishable, statistically significant increases in the relative abundance of 29 and decreases in 25 RNAs were identified by RNA microarray analysis. Of RNAs whose expression was consistently altered by the absence of CFTR, functional classes of genes influencing gene transcription, inflammation, intracellular trafficking, signal transduction, and ion transport were identified. RNAs encoding the transcription factor CCAAT enhancer-binding protein (CEBP) delta and interleukin (IL) 1beta, both known to regulate CFTR expression, were induced, perhaps indicating adaptation to the lack of CFTR. RNAs mediating lung inflammation including calgranulin-S100 family members, IL-1beta and IL-4, were increased. Likewise, expression of several membrane transport proteins that interact directly with CFTR were increased, suggesting that CFTR-protein complexes initiate genomic responses. Absence of CFTR influenced the expression of genes modulating diverse pulmonary cell functions that may ameliorate or contribute to the pathogenesis of CF. Keywords: Genotype comparison
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Overall design |
Lungs from sex-matched littermates at 3, 6, and 11 weeks of agewere carefully dissected and the conducting airways and mediastinal structures removed.
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Citation missing |
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Submission date |
Mar 21, 2006 |
Last update date |
Feb 18, 2018 |
Contact name |
Yan Xu |
E-mail(s) |
[email protected]
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Phone |
513-6368921
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Organization name |
Cincinnati Children's Hospital Medical Center
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Street address |
3333 Burnet Ave
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City |
Cincinnati |
State/province |
OH |
ZIP/Postal code |
45229 |
Country |
USA |
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Platforms (2) |
GPL32 |
[MG_U74A] Affymetrix Murine Genome U74A Array |
GPL81 |
[MG_U74Av2] Affymetrix Murine Genome U74A Version 2 Array |
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Samples (12)
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Relations |
BioProject |
PRJNA94517 |
Supplementary data files not provided |
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