Article
Physiologic and molecular consequences of endothelial Bmpr2 mutation.
Respiratory research - 22 Jun 2011
Majka Susan, Hagen Moira, Blackwell Thomas, Harral Julie, Johnson Jennifer A, Gendron Robert, Paradis Helene, Crona Daniel, Loyd James E, Nozik-Grayck Eva, Stenmark Kurt R, West James
Abstract excerpt
BACKGROUND: 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. METHODS: In vivo experiments were performed on adult mice with conditional endothelial-specific expression of...
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