Tatius, Bintang and Wasityastuti, Widya and Astarini, Fajar Dwi and Nugrahaningsih, Dwi Aris Agung (2021) Significance of BMPR2 mutations in pulmonary arterial hypertension. Respiratory Investigation, 59 (4). 397 – 407. ISSN 22125345
Full text not available from this repository. (Request a copy)Abstract
Pulmonary arterial hypertension (PAH) is a debilitating disease that results from progressive remodeling and inflammation of pulmonary arteries. PAH develops gradually, is difficult to diagnose, and has a high mortality rate. Although mutation in the bone morphogenetic protein receptor 2 (BMPR2) gene has been identified as the main genetic cause of PAH, the underlying pathways involving the pathophysiology of PAH are complex and still not fully understood. Endothelial dysfunction has been observed in PAH development that results in a multitude of disturbances in the cellular processes in pulmonary vessels. Changes in the pulmonary vasculature caused by the disruption of BMPR2 signaling are observed in three main vascular components; endothelial cells, smooth muscle cells, and fibroblasts. BMPR2 also has a prominent role in maintenance of the immune system. The disruption of BMPR2 signaling pathway causes an increased degree of inflammation and decreases the ability of the immune system to resolve it. Inflammatory processes and changes in pulmonary vasculature interact with one another, resulting in the progression of chronic PAH. In this review, we highlight the various components of vascular remodeling and immune response that are caused by disruption of BMPR2 signaling, including the clinical evidence and the prospects of these components as a potential target for PAH therapy. Indeed, development of drugs to target the pathogenic pathways involved in PAH may complement existing treatment regimens and improve patient prognosis. © 2021 The Japanese Respiratory Society
Item Type: | Article |
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Additional Information: | Cited by: 17 |
Uncontrolled Keywords: | Bone Morphogenetic Protein Receptors, Type II; Endothelial Cells; Humans; Mutation; Pulmonary Arterial Hypertension; Pulmonary Artery; bone morphogenetic protein receptor 2; cyclic GMP dependent protein kinase; endothelial nitric oxide synthase; fibroblast growth factor 2; high mobility group protein; immunoglobulin enhancer binding protein; intercellular adhesion molecule 1; interleukin 6; mitogen activated protein kinase p38; peroxisome proliferator activated receptor gamma; transforming growth factor beta1; tumor necrosis factor; BMPR2 protein, human; bone morphogenetic protein receptor 2; BPAEC cell line; endothelial dysfunction; epithelial mesenchymal transition; human; lung blood vessel; pulmonary hypertension; Review; vascular remodeling; endothelium cell; genetics; mutation; pulmonary artery |
Subjects: | R Medicine > RB Biomedical Sciences |
Divisions: | Faculty of Medicine, Public Health and Nursing > Biomedical Sciences |
Depositing User: | Sri JUNANDI |
Date Deposited: | 26 Sep 2024 06:25 |
Last Modified: | 26 Sep 2024 06:25 |
URI: | https://ir.lib.ugm.ac.id/id/eprint/4628 |