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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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Autori principali: | , , , , , |
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Natura: | Artigo |
Lingua: | Inglês |
Pubblicazione: |
Oxford University Press
2013
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Accesso online: | 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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