American journal of respiratory and critical care medicine
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Am. J. Respir. Crit. Care Med. · Oct 2015
BMPR-II Deficiency Promotes Pulmonary Hypertension via Increased Inflammatory Cytokine Production.
Mutations in bone morphogenetic protein receptor type II (BMPR-II) underlie most cases of heritable pulmonary arterial hypertension (PAH). However, disease penetrance is only 20-30%, suggesting a requirement for additional triggers. Inflammation is emerging as a key disease-related factor in PAH, but to date there is no clear mechanism linking BMPR-II deficiency and inflammation. ⋯ This study demonstrates that BMPR-II deficiency promotes an exaggerated inflammatory response in vitro and in vivo, which can instigate development of pulmonary hypertension.