GEO DataSets

Analysis of lung from transgenics expressing a BMPR2 tail domain mutation (BMPR2R899X) in smooth muscle. Nine weeks of BMPR2R899X expression can result in elevated right ventricular systolic pressures (RVSP), producing a mutant phenotype relevant to human pulmonary arterial hypertension (PAH).

Organism:

Mus musculus

Type:

Expression profiling by array, count, 3 disease state sets

Platform:

GPL1261

Series:

GSE11018

6 Samples

Download data: CEL, CHP

(Submitter supplied) Familial pulmonary arterial hypertension (fPAH) is associated with mutations in BMPR2. Many of these mutations occur in the BMPR2 tail domain, leaving the SMAD functions intact. In order to determine the in vivo consequences of BMPR2 tail domain mutation, we created a smooth-muscle specific doxycycline inducible BMPR2 mutation with an arginine to termination mutation at amino acid 899. When these SM22-rtTA x TetO7-BMPR2R899X mice had transgene induced for 9 weeks, starting at 4 weeks of age, they universally developed pulmonary vascular pruning as assessed by fluorescent microangiography. more...

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS3799

Platform:

GPL1261

6 Samples

Download data: CEL, CHP

(Submitter supplied) Pulmonary arterial hypertension (PAH) is thought to be driven by dysfunction of pulmonary vascular microendothelial cells (PMVEC). Most hereditary PAH is associated with BMPR2 mutations. However, the physiologic and molecular consequences of expression of BMPR2 mutations in PMVEC are unknown.

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS4543

Platform:

GPL6246

6 Samples

Download data: CEL

Analysis of PMVECs cultured from triple transgenics carrying immortomouse gene, a transactivator, and either control, Bmpr2delx4+ or Bmpr2R899X mutation. Hereditary pulmonary arterial hypertension is often associated with BMPR2 mutations. Results provide insight into role of Bmpr2 in endothelium.

Organism:

Mus musculus

Type:

Expression profiling by array, transformed count, 3 genotype/variation sets

Platform:

GPL6246

Series:

GSE28043

6 Samples

Download data: CEL

(Submitter supplied) BMPR2 mutation causes pulmonary arterial hypertension (PAH); ACE2 treatment can resolve established BMPR2-mediated PAH. The purpose of this study was to uncover the molecular mechanism behind this.

Organism:

Mus musculus

Type:

Expression profiling by array

Platform:

GPL1261

8 Samples

Download data: CEL, CHP

(Submitter supplied) Mice expressing a doxycycline-inducible dominant negative BMPR2 transgene expressed only in smooth muscle are activated for one or eight weeks, and compared to transactivator-only mice also fed doxycycline. All mice are 12 weeks of old at sacrifice. Keywords: time course with SM22-rtTA x tetO7-BMPR2 transgene

Organism:

Mus musculus

Type:

Expression profiling by array

Dataset:

GDS2147

Platform:

GPL1261

6 Samples

Download data

Analysis of lungs 1 or 8 weeks following induction in smooth muscle of a dominant negative mutant form of type 2 bone morphogenic protein receptor (BMPR2). Results provide insight into the pathogenesis of hereditary pulmonary arterial hypertension (PAH), a disorder associated with BMPR2 mutations.

Organism:

Mus musculus

Type:

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

Platform:

GPL1261

Series:

GSE5255

6 Samples

Download data

(Submitter supplied) Gene expression in the right ventricle is different in control patients as compared to either idiopathic dilated cardiomyopathy or pulmonary arterial hypertension

Organism:

Homo sapiens

Type:

Expression profiling by array

Dataset:

GDS5610

Platform:

GPL6244

6 Samples

Download data: CEL

Analysis of biopsies of right ventricle (RV) from patients with pulmonary arterial hypertension (PAH) with bone morphogenetic protein receptor type II (BMPR2) mutation. Results provide insight into molecular mechanisms underlying the metabolic effects of mutant BMPR2 in PAH RVs.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 3 disease state sets

Platform:

GPL6244

Series:

GSE67492

6 Samples

Download data: CEL

(Submitter supplied) Bmpr2 mutations are critical risk factors for hereditary pulmonary arterial hypertension (hPAH) with approximately 20% of carriers developing disease. There is an unmet medical need to understand how environmental factors, such as inflammation, render Bmpr2 mutants susceptible to PAH. Overexpressing 5-lipoxygenase (5-LO) provokes lung inflammation and transient PAH in Bmpr2+/- mice. Accordingly, 5-LO and its metabolite, leukotriene B4 (LTB4), are candidates for the ‘second hit’. more...

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL20301

18 Samples

Download data: TXT

(Submitter supplied) The goal of this study is to compare transcriptome profiling (RNA-seq) in controls, unaffected BMPR2 mutation carriers and affected familial pulmonary arterial hypertension patients, to elucidate a protective feature in iPS derived endothelial cells from the mutation carriers.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL11154

11 Samples

Download data: TXT

(Submitter supplied) Pulmonary arterial hypertension (PAH) is a fatal disease characterized by a proliferative endothelial cell phenotype, inflammation and pulmonary vascular remodeling. BMPR2 loss-of-function has been linked to pathologic plexiform lesions with obliteration of distal pulmonary arteries distal pulmonary arteries BMPR2 silencing inprimary human pulmonary artery ECs (HPAECs) recapitulate important aspects of cellular dysfunction and deregulated signaling associated with PAH.

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL15207

16 Samples

Download data: CEL

(Submitter supplied) Endothelial cell (EC) dysfunction plays a key role in the pathogenesis of pulmonary arterial hypertension (PAH). To avoid cell cultures and whole lung tissue samples, we have, for the first time, used CD31 antibody coated magnetic beads in conjunction with genome scale RNA expression microarrays to profile ECs in vivo at any stage of PAH. We hypothesized that targeting early stages of the disease would identify novel mediators of PAH and genes linked to bone morphogenetic protein receptor 2 (BMPR2) signaling.

Organism:

Rattus norvegicus

Type:

Expression profiling by array

Platform:

GPL14797

8 Samples

Download data: TXT

(Submitter supplied) Vascular remodeling in pulmonary arterial hypertension (PAH) involves proliferation and migration of endothelial and smooth muscle cells, leading to obliterative vascular lesions. Previous studies have indicated that the endothelial cell proliferation is quasi-neoplastic, with evidence of monoclonality and instability of short DNA microsatellite sequences. To assess whether there is larger scale genomic instability, we performed genome-wide microarray copy number analysis on pulmonary artery endothelial (PAEC) and smooth muscle cells isolated from the lungs of PAH patients. more...

Organism:

Homo sapiens

Type:

Genome variation profiling by SNP array; SNP genotyping by SNP array

Platforms:

GPL5677 GPL6985 GPL8887

24 Samples

Download data: IDAT, TXT

(Submitter supplied) Vascular remodeling in pulmonary arterial hypertension (PAH) involves proliferation and migration of endothelial and smooth muscle cells, leading to obliterative vascular lesions. Previous studies have indicated that the endothelial cell proliferation is quasi-neoplastic, with evidence of monoclonality and instability of short DNA microsatellite sequences. To assess whether there is larger scale genomic instability, we performed genome-wide microarray copy number analysis on pulmonary artery endothelial (PAEC) and smooth muscle cells isolated from the lungs of PAH patients. more...

Organism:

Homo sapiens

Type:

Genome variation profiling by SNP array; SNP genotyping by SNP array

Platforms:

GPL6801 GPL3720 GPL3718

37 Samples

Download data: CEL, CHP