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Physiology in Medicine review article. Pulmonary arterial hypertension (PAH) occurs as an idiopathic disease (formerly called primary pulmonary hypertension) and as a consequence of other illnesses. Inherited susceptibility to PAH occurs in families and is almost always due to mutations in genes of the TGF-Beta family of receptors. Many signaling systems have been found to participate in PAH, including K channels, serotonin, angiopoietin, and cyclooxygenases. An interaction of these signaling systems with BMPR2 is a focus of research in PAH. Approaches to altering the imbalance of activation of BMPR2 and other TGF-Beta receptors may yield future therapies for PAH.
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