GEO DataSets

(Submitter supplied) Cystic Fibrosis (CF) is associated with pathology in multiple tissues including the lung, digestive tract and reproductive system. Lung disease is primarily a post-natal event but other organs are affected before birth. Here we use the CF sheep model to investigate the initiation and progression of CF disease through gestation.

Organism:

Ovis aries

Type:

Expression profiling by high throughput sequencing

Platform:

GPL27721

20 Samples

Download data: TSV

(Submitter supplied) Cystic fibrosis (CF) is a recessive disease caused by mutations in theCF transmembrane conductance regulator(CFTR) gene. The most common symptoms include progressive lung disease and chronic digestive conditions. CF is the first human genetic disease to benefit from having five different species of animal models. Despite the phenotypic differences among the animal models and human CF, these models have provided invaluable insight into understanding disease mechanisms at the organ-system level. more...

Organism:

Drosophila melanogaster

Type:

Expression profiling by high throughput sequencing

Platform:

GPL19132

4 Samples

Download data: CSV

(Submitter supplied) Background: Cystic fibrosis (CF) is caused by mutations in the CFTR gene that impair function of this cAMP-regulated Cl- channel. In the small intestine, loss of CFTR function creates a dehydrated, acidic luminal environment which is believed to cause an accumulation of mucus, a phenotype characteristic of CF. CF mice have an innate immune response and impaired intestinal transit as well. We investigated whether lubiprostone, which activates the CLC2 Cl- channel, would improve the CF intestinal phenotype. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS4251

Platform:

GPL1261

4 Samples

Download data: CEL, CHP

Analysis of small intestine from Cftr null and wild type mice treated with fluid secretion activator lubiprostone. Cystic fibrosis (CF) mice display CF trait of impaired mucus turnover. Results provide insight into response of CF intestine to lubiprostone treatment.

Organism:

Mus musculus

Type:

Expression profiling by array, count, 2 agent, 2 genotype/variation sets

Platform:

GPL1261

Series:

GSE18327

4 Samples

Download data: CEL, CHP

(Submitter supplied) Dysfunction of the cystic fibrosis transmembrane regulator (CFTR) in cystic fibrosis (CF) results in exaggerated and chronic inflammation as well as increased susceptibility to chronic pulmonary infections, in particular with Pseudomonas aeruginosa. Based on the concept that host immune responses do not seem to be adequate to eradicate P.aeruginosa from the lungs of CF patients and that dendritic cells (DC) play an important role in initiating and shaping adaptive immune responses, this study analyzed the role of CFTR in bone marrow-derived murine DC from CFTR knockout (CF) mice with and without exposure to P.aeruginosa. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL81

24 Samples

Download data: CEL