Article
Bone morphogenetic protein receptor type II deficiency and increased inflammatory cytokine production. A gateway to pulmonary arterial hypertension.
American journal of respiratory and critical care medicine - 1 Oct 2015
Soon Elaine, Crosby Alexi, Southwood Mark, Yang Peiran, Tajsic Tamara, Toshner Mark, Appleby Sarah, Shanahan Catherine M, Bloch Kenneth D, Pepke-Zaba Joanna, Upton Paul, Morrell Nicholas W
Abstract excerpt
RATIONALE: 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. OBJECTIVES: To...
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