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Lookup NU author(s): Dr Jenny Grant
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Pulmonary arterial remodeling is a presently irreversible pathologic hallmark of pulmonary arterial hypertension (PAH). This complex disease involves pathogenic dysregulation of all cell types within the small pulmonary arteries contributing to vascular remodeling leading to intimal lesions, resulting in elevated pulmonary vascular resistance and right heart dysfunction. Mutations within the bone morphogenetic protein receptor 2 gene, leading to dysregulated proliferation of pulmonary artery smooth muscle cells, have been identified as being responsible for heritable PAH. Indeed, the disease is characterized by excessive cellular proliferation and resistance to apoptosis of smooth muscle and endothelial cells. Significant gene dysregulation at the transcriptional and signaling level has been identified. MicroRNAs are small non-coding RNA molecules that negatively regulate gene expression and have the ability to target numerous genes, therefore potentially controlling a host of gene regulatory and signaling pathways. The major role of miRNAs in pulmonary arterial remodeling is still relatively unknown although research data is emerging apace. Modulation of miRNAs represents a possible therapeutic target for altering the remodeling phenotype in the pulmonary vasculature. This review will focus on the role of miRNAs in regulating smooth muscle and endothelial cell phenotypes and their influence on pulmonary remodeling in the setting of PAH.
Author(s): Grant JS, White K, MacLean MR, Baker AH
Publication type: Article
Publication status: Published
Journal: Cellular and Molecular Life Sciences
Year: 2013
Volume: 70
Issue: 23
Pages: 4479-4494
Print publication date: 01/12/2013
Online publication date: 06/06/2013
Acceptance date: 22/05/2013
ISSN (print): 1420-682X
ISSN (electronic): 1420-9071
Publisher: Springer
URL: http://dx.doi.org/10.1007/s00018-013-1382-5
DOI: 10.1007/s00018-013-1382-5
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