Transcriptomics

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Bmpr2 mutation in murine PMVEC


ABSTRACT: 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 were isolated from triple transgenic mice carrying the immortomouse gene, a transactivator, and either control, Bmpr2delx4+ or Bmpr2R899X mutation

ORGANISM(S): Mus musculus  

SUBMITTER: Tom Blackwell   James West 

PROVIDER: E-GEOD-28043 | ArrayExpress | 2011-07-01

SECONDARY ACCESSION(S): GSE28043PRJNA139753

REPOSITORIES: GEO, ArrayExpress

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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.In vivo experiments were performed on adult mice with conditional endothelial-specific expression of the truncation mutation Bmpr2delx4+, with age-matched transactivator-only mice as controls. Phenotype was  ...[more]

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