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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. PMVEC ...
ORGANISM(S): Mus musculus 
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...
ORGANISM(S): Homo sapiens 
Upon ligand binding, bone morphogenetic protein (BMP) receptors form active tetrameric complexes, comprised of two type I and two type II receptors, which then transmit signals to SMAD proteins. The link between receptor tetramerization and the mechanism of kinase activation, however, has not been e...
ORGANISM(S): Homo sapiens (Human) 
2021-08-16 | PXD022944 | Pride
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. Experiment Overall Design: Each chip is a pool of ...
ORGANISM(S): Mus musculus 
Background: While BMPR2 mutation strongly predisposes to pulmonary arterial hypertension (PAH), only 20% of mutation carriers develop clinical disease. This finding suggests that modifier genes contribute to FPAH clinical expression. Since modifiers are likely to be common alleles, this problem is n...
ORGANISM(S): Homo sapiens 
Proteome analysis of BMPR2 mutation PASMCs with or without PERK knockout under hypoxia.
ORGANISM(S): Mus Musculus (mouse) 
Breast Cancer Reveals Latent BMPR2-Related Susceptibility to Pulmonary Hypertension
BMPR2 Regulates Hypoxic Extracellular Vesicle Signaling to Maintain Vascular Homeostasis.
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. Four groups: +/- ACE2 and +/- BMPR2 transgene, two arrays each, each array a pool of three animals.
ORGANISM(S): Mus musculus 
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 ...
ORGANISM(S): Rattus norvegicus 
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