Pulmonary arterial hypertension and BMP system abnormality

Research output: Contribution to journalArticle

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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.

Original languageEnglish
Pages (from-to)2083-2090
Number of pages8
JournalNihon rinsho. Japanese journal of clinical medicine
Volume66
Issue number11
Publication statusPublished - Nov 2008

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Pulmonary Hypertension
Smooth Muscle Myocytes
Mutation
Pulmonary Artery
Type II Bone Morphogenetic Protein Receptors
Cell Proliferation
Germ-Line Mutation
Transforming Growth Factor beta
Endothelial Cells
Apoptosis
Ligands
Wounds and Injuries

ASJC Scopus subject areas

  • Medicine(all)

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Pulmonary arterial hypertension and BMP system abnormality. / Otsuka, Fumio.

In: Nihon rinsho. Japanese journal of clinical medicine, Vol. 66, No. 11, 11.2008, p. 2083-2090.

Research output: Contribution to journalArticle

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