The lysosomal inhibitor, chloroquine, increases cell surface BMPR-II levels and restores BMP9 signalling in endothelial cells harbouring BMPR-II mutations

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 resul...

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Bibliographic Details
Main Authors: Dunmore, Benjamin J., Drake, Kylie M., Upton, Paul D., Toshner, Mark R., Aldred, Micheala A., Morrell, Nicholas W.
Format: Artigo
Language:Inglês
Published: Oxford University Press 2013
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Articles
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC3749859/
https://ncbi.nlm.nih.gov/pubmed/23669347
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddt216
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