Article
BMPR2 gene delivery reduces mutation-related PAH and counteracts TGF-β-mediated pulmonary cell signalling.
Respirology (Carlton, Vic.) - 1 Apr 2016
Feng Feng, Harper Rebecca L, Reynolds Paul N
Abstract excerpt
BACKGROUND AND OBJECTIVE: Idiopathic, familial and secondary pulmonary arterial hypertension (PAH) are associated with reduced bone morphogenetic protein receptor type 2 (BMPR2) expression, and in some contexts, TGF-β upregulation. Our aims were to assess BMPR2 gene therapy in a PAH mouse model and to assess the impact on TGF-β signalling. METHODS: Using a targeted in vivo gene delivery approach, we assessed the...
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