title: [Pulmonary arterial hypertension and BMP system abnormality].
abstract: Genetic analysis has uncovered that familial and idiopathic pulmonary arterial hypertension (PAH) is linked to germline mutations in BMP type II receptor (BMPRII). PAH is characterized by enhanced remodeling of pulmonary arteries due to arterial smooth muscle cell proliferation. BMPRII mutations contribute to abnormal mitotic responses to BMP ligands in pulmonary artery smooth muscle cells. Unbalanced Smad signaling induced by BMP and TGFbeta is functionally involved in the pathogenesis of PAH. BMPRII mutations also increase the susceptibility of endothelial cell apoptosis. The combination of increased endothelial injury and impaired suppression of smooth muscle cell proliferation is critical for the cellular pathogenesis of PAH. However, the detailed molecular mechanism leading to severe vascular remodeling caused by BMPRII mutations has yet to be elucidated.
authors.affiliation: Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences.
authors.forename: Fumio
authors.initials: F
authors.lastname: Otsuka
mesh_terms: D052004:Bone Morphogenetic Protein Receptors / Q000235:genetics / Q000502:physiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000235:genetics; D009154:Mutation; D015398:Signal Transduction
chemical_list: D052004:Bone Morphogenetic Protein Receptors