At the X-Roads of Sex and Genetics in Pulmonary Arterial Hypertension

Group 1 pulmonary hypertension (pulmonary arterial hypertension; PAH) is a rare disease characterized by remodeling of the small pulmonary arteries leading to progressive elevation of pulmonary vascular resistance, ultimately leading to right ventricular failure and death. Deleterious mutations in t...

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Veröffentlicht in:Genes 2020-11, Vol.11 (11), p.1371
Hauptverfasser: Cirulis, Meghan M., Dodson, Mark W., Brown, Lynn M., Brown, Samuel M., Lahm, Tim, Elliott, Greg
Format: Artikel
Sprache:eng
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Zusammenfassung:Group 1 pulmonary hypertension (pulmonary arterial hypertension; PAH) is a rare disease characterized by remodeling of the small pulmonary arteries leading to progressive elevation of pulmonary vascular resistance, ultimately leading to right ventricular failure and death. Deleterious mutations in the serine-threonine receptor bone morphogenetic protein receptor 2 (BMPR2; a central mediator of bone morphogenetic protein (BMP) signaling) and female sex are known risk factors for the development of PAH in humans. In this narrative review, we explore the complex interplay between the BMP and estrogen signaling pathways, and the potentially synergistic mechanisms by which these signaling cascades increase the risk of developing PAH. A comprehensive understanding of these tangled pathways may reveal therapeutic targets to prevent or slow the progression of PAH.
ISSN:2073-4425
2073-4425
DOI:10.3390/genes11111371