Article
The lysosomal inhibitor, chloroquine, increases cell surface BMPR-II levels and restores BMP9 signalling in endothelial cells harbouring BMPR-II mutations.
Human molecular genetics - 15 Sept 2013
Dunmore Benjamin J, Drake Kylie M, Upton Paul D, Toshner Mark R, Aldred Micheala A, Morrell Nicholas W
Abstract excerpt
Pulmonary arterial hypertension (PAH) is characterized by dysregulated pulmonary artery endothelial cell (PAEC) proliferation, apoptosis and permeability. Loss-of-function mutations in the bone morphogenetic protein receptor type-II (BMPR-II) are the most common cause of heritable PAH, usually re...
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