Altered expression and signal transduction of endothelin-1 receptors in heritable and idiopathic pulmonary arterial hypertension.

ABSTRACT: Human pulmonary arterial smooth muscle cells (PASMC) were isolated from elastic pulmonary arteries dissected from lungs of individuals with and without pulmonary arterial hypertension (PAH). Reflecting increased smooth muscle constriction in cells from PAH subject, Ca(2+) influx in response to endothelin-1 (ET-1) increased in all the PAH PASMC populations relative to the normal donor control cells. The ETA receptor mRNA levels remained unchanged, whereas the ETB receptor mRNA levels decreased in both heritable and idiopathic PAH-derived PASMC. All the PASMC populations expressed considerably higher ETA compared to ETB receptor number. Both ETA and ETB receptor numbers were reduced in bone morphogenetic protein receptor type II (BMPR2) mutation PAH. ETB receptors showed a particular reducti

SUBMITTER: Yu J 

PROVIDER: S-EPMC3496420 | biostudies-literature | 2013 Feb

REPOSITORIES: biostudies-literature