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Series GSE4513 Query DataSets for GSE4513
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
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
 
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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Submission date Mar 21, 2006
Last update date Feb 18, 2018
Contact name Yan Xu
E-mail(s) [email protected]
Phone 513-6368921
Organization name Cincinnati Children's Hospital Medical Center
Street address 3333 Burnet Ave
City Cincinnati
State/province OH
ZIP/Postal code 45229
Country USA
 
Platforms (2)
GPL32 [MG_U74A] Affymetrix Murine Genome U74A Array
GPL81 [MG_U74Av2] Affymetrix Murine Genome U74A Version 2 Array
Samples (12)
GSM101267 KO_2
GSM101274 WT_2
GSM101275 KO_2b
Relations
BioProject PRJNA94517

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