GEO DataSets

(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

(Submitter supplied) Gene expression profiling with microarrays was used to identify genes differentially expressed in the lungs of B6 and BALB CF mice compared to non-CF littermates Keywords: disease state analysis

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS1941

Platform:

GPL1261

23 Samples

Download data: CEL

Analysis of lungs of C57BL/6J (B6) or BALB/c (BALB) animals deficient for cystic fibrosis transmembrane conductance regulator (CFTR). B6 background animals have a higher propensity to develop CF lung disease. Results suggest the variable severity of CF is controlled by multiple genetic factors.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 2 gender, 2 genotype/variation, 2 strain sets

Platform:

GPL1261

Series:

GSE3100

23 Samples

Download data: CEL

(Submitter supplied) Wild-type and dF508 CFTR mutant human bronchial epithelial cells were uninfected or infected for 3 hours at an MOI of 30-50 with P.aeruginosa strain PAO1. Each cell line was tested in 4 replicates. Each replicate consists of 4 data sets: 2 sets of duplicate spots on 2 arrays. The two arrays in each replicate were dye-swapped pairs where the opposite dye was used for each sample in the second array. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL1883

32 Samples

Download data

(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) We used microarrays to look at differential gene expression in PBMC samples from proband, proband's parents, and healthy controls We used microarrays to look at the differential gene expression in THP-1 monocyte/macrophages after incubation with plasma from CF probands, proband's parents, healthy controls

Organism:

Homo sapiens; synthetic construct

Type:

Expression profiling by array; Non-coding RNA profiling by array

Platforms:

GPL23126 GPL21572

35 Samples

Download data: CEL, CHP

(Submitter supplied) This study performed single-cell RNA sequencing (scRNA-seq) analyses on lung-lavaged cells from macrophage-specific cystic fibrosis (Mac-CF) mice and congenic wild-type (WT) mice that had been challeged with Pseudomonas aeruginsa (PsA) for different time points (1, 3 and 5 days). Differences in global gene transcription were interrogated and compared. Results demonstrate that CFTR loss of function in macrophages altered the cell transcriptional program that mostly affected mitochondrial respiration, immune responses, and cellular antioxidant system. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL30172

4 Samples

Download data: H5, TXT

(Submitter supplied) The differentiation of pluripotent stem cells has been broadly used in studying the disease mechanism and development process. We previously described a method for differentiating human pluripotent stem cells (hPSCs) into salivary gland epithelial progenitors (SGEPs). Here, to investigate whether the hPSCs-derived SGEPs are capable of modeling the characteristics of cystic fibrosis (CF), a disease impact SG function caused by mutation of cystic fibrosis transmembrane conductance regulator (CFTR) gene, the CFTR knockout hPSCs were differentiated into CF-SGEPs using the same protocol. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24676

6 Samples

Download data: TXT

(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