Nihon Rinsho. 2008 Nov;66(11):2083-90.

[Pulmonary arterial hypertension and BMP system abnormality].

[Article in Japanese]

Source

Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences.

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.

PMID:: 19051724; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms, Substances

Publication Types

MeSH Terms

Substances

Bone Morphogenetic Protein Receptors

LinkOut - more resources

Medical

Pulmonary Hypertension - MedlinePlus Health Information

Supplemental Content

Save items

Related citations in PubMed

Involvement of the bone morphogenetic protein system in endothelin- and aldosterone-induced cell proliferation of pulmonary arterial smooth muscle cells isolated from human patients with pulmonary arterial hypertension. [Hypertens Res. 2010]
Involvement of the bone morphogenetic protein system in endothelin- and aldosterone-induced cell proliferation of pulmonary arterial smooth muscle cells isolated from human patients with pulmonary arterial hypertension.
Yamanaka R, Otsuka F, Nakamura K, Yamashita M, Otani H, Takeda M, Matsumoto Y, Kusano KF, Ito H, Makino H. Hypertens Res. 2010 May; 33(5):435-45. Epub 2010 Feb 26.
Dysfunctional Smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension. [Circ Res. 2005]
Dysfunctional Smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension.
Yang X, Long L, Southwood M, Rudarakanchana N, Upton PD, Jeffery TK, Atkinson C, Chen H, Trembath RC, Morrell NW. Circ Res. 2005 May 27; 96(10):1053-63. Epub 2005 Apr 21.
Receptor for activated C-kinase 1, a novel interaction partner of type II bone morphogenetic protein receptor, regulates smooth muscle cell proliferation in pulmonary arterial hypertension. [Circulation. 2007]
Receptor for activated C-kinase 1, a novel interaction partner of type II bone morphogenetic protein receptor, regulates smooth muscle cell proliferation in pulmonary arterial hypertension.
Zakrzewicz A, Hecker M, Marsh LM, Kwapiszewska G, Nejman B, Long L, Seeger W, Schermuly RT, Morrell NW, Morty RE, et al. Circulation. 2007 Jun 12; 115(23):2957-68. Epub 2007 May 21.
Review Pulmonary hypertension due to BMPR2 mutation: a new paradigm for tissue remodeling? [Proc Am Thorac Soc. 2006]
Review Pulmonary hypertension due to BMPR2 mutation: a new paradigm for tissue remodeling?
Morrell NW. Proc Am Thorac Soc. 2006 Nov; 3(8):680-6.
Review Role of bone morphogenetic protein receptors in the development of pulmonary arterial hypertension. [Adv Exp Med Biol. 2010]
Review Role of bone morphogenetic protein receptors in the development of pulmonary arterial hypertension.
Morrell NW. Adv Exp Med Biol. 2010; 661:251-64.

See reviews... See all...

Recent activity

Clear Turn Off Turn On

[Pulmonary arterial hypertension and BMP system abnormality].
[Pulmonary arterial hypertension and BMP system abnormality].
Nihon Rinsho. 2008 Nov ;66(11):2083-90.

PubMed
Brain dysfunction during motor activation and corpus callosum alterations in sch...
Brain dysfunction during motor activation and corpus callosum alterations in schizophrenia measured by cerebral blood flow and magnetic resonance imaging.
Biol Psychiatry. 1991 Mar 15 ;29(6):535-55.

PubMed
Neurology referrals to a liaison psychiatry service.
Neurology referrals to a liaison psychiatry service.
Ir Med J. 2008 Oct ;101(9):271-3.

PubMed
Basic and therapeutic trial results obtained in the spontaneous AK leukemia (lym...
Basic and therapeutic trial results obtained in the spontaneous AK leukemia (lymphoma) model-end of 1971.
Cancer Chemother Rep. 1972 Jun ;56(3):273-87, 289-314.

PubMed
Effects of an EDTA infusion on the urinary elimination of several elements in he...
Effects of an EDTA infusion on the urinary elimination of several elements in healthy subjects.
Br J Clin Pharmacol. 1991 Mar ;31(3):347-9.

PubMed

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

PubMed

Result Filters

Display Settings:

Send to:

[Pulmonary arterial hypertension and BMP system abnormality].

Source

Abstract

Publication Types, MeSH Terms, Substances

Publication Types

MeSH Terms

Substances

LinkOut - more resources

Medical

Supplemental Content

Save items

Related citations in PubMed

Recent activity

Simple NCBI Directory

Getting Started

Resources

Popular

Featured

NCBI Information