GEO DataSets

Analysis of cells that constitutively express misfolded surfactant protein C, which induces chronic endoplasmic reticulum (ER) stress and cytotoxicity. Results provide evidence for an NF-kB-dependent adaptive response to the chronic ER stress imposed by the misfolded protein.

Organism:

Homo sapiens

Type:

Expression profiling by array, count, 3 agent sets

Platform:

GPL97

Series:

GSE2980

9 Samples

Download data: CEL

(Submitter supplied) Mutations in the gene encoding surfactant protein C (SFTPC) have been linked to interstitial lung disease in children and adults. Expression of the index mutation, SP-Cdeltaexon4, in transiently transfected cells and type II cells of transgenic mice resulted in misfolding of the proprotein, activation of ER stress pathways and cytotoxicity. In the current study we show that stably transfected cells adapted to chronic ER stress imposed by constitutive expression of SP-Cdeltaexon4 via an NF-kB-dependent pathway. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Datasets:

GDS1841 GDS2054

Platforms:

GPL97 GPL96

18 Samples

Download data: CEL

Analysis of cells that constitutively express misfolded surfactant protein C, which induces chronic endoplasmic reticulum (ER) stress and cytotoxicity. Results provide evidence for an NF-kB-dependent adaptive response to the chronic ER stress imposed by the misfolded protein.

Organism:

Homo sapiens

Type:

Expression profiling by array, count, 3 agent sets

Platform:

GPL96

Series:

GSE2980

9 Samples

Download data: CEL

(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL18573

7 Samples

Download data: H5

(Submitter supplied) We performed transcriptomic profiling of cells derived from human induced pluripotent stem cells (iPSCs) using our previously described distal lung directed differentiation protocol to generate alveolar epithelial type 2 cells (iAEC2s). We used the SPC2 human iPSC line and specifically the SPC2-ST-B2 (SFTPCtdT/WT) and SPC2-ST-C11 (SFTPCI73T/tdT) clones containing a SFTPCtdTomato knock-in reporter. SFTPCtdTomato+ cells were sorted on day 51 of differentiation and maintained without further sorting in self-renewing 3D alveolosphere cultures passaged approximately every 2 weeks through day 114. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL18573

3 Samples

Download data: H5

(Submitter supplied) We performed transcriptomic profiling of cells derived from human induced pluripotent stem cells (iPSCs) using our previously described distal lung directed differentiation protocol to generate alveolar epithelial type 2 cells (iAEC2s). We used the SPC2 human iPSC line and specifically the SPC2-ST-B2 (SFTPCtdT/WT) and SPC2-ST-C11 (SFTPCI73T/tdT) clones containing a SFTPCtdTomato knock-in reporter. SFTPCtdTomato+ cells were sorted on day 41 and again on day 79 of differentiation. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL18573

2 Samples

Download data: H5

(Submitter supplied) We performed transcriptomic profiling of cells derived from human induced pluripotent stem cells (iPSCs) using our previously described distal lung directed differentiation protocol to generate alveolar epithelial type 2 cells (iAEC2s). We used the SPC2 human iPSC line and specifically the SPC2-ST-B2 (SFTPCtdT/WT) and SPC2-ST-C11 (SFTPCI73T/tdT) clones containing a SFTPCtdTomato knock-in reporter. Live SFTPCtdTomato+ iAEC2s were sorted on day 30 and encapsulated for scRNA-seq using the 10X Chromium System. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL18573

2 Samples

Download data: H5

(Submitter supplied) Mutations in the gene encoding surfactant protein C (SFTPC) are associated with interstitial lung disease in children and adults. To assess the natural history of disease, we knocked-in a familial disease-associated SFTPC mutation, L188Q (L184Q in mice), into the mouse Sftpc locus. Expression of L184Q (LQ) resulted in formation of an unstable, misfolded proprotein (proSP-CLQ) that was rapidly degraded by the proteasome pathway. more...

Organism:

Mus musculus

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24247

4 Samples

Download data: TAR