2020
DOI: 10.1530/vb-19-0030
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Targeting epigenetic mechanisms as an emerging therapeutic strategy in pulmonary hypertension disease

Abstract: Pulmonary arterial hypertension (PAH) is a multifactorial cardiopulmonary disease characterized by an elevation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR), which can lead to right ventricular (RV) failure, multi-organ dysfunction, and ultimately to premature death. Despite the advances in molecular biology, the mechanisms underlying pulmonary hypertension (PH) remain unclear. Nowadays, there is no curative treatment for treating PH. Therefore, it is crucial to identify novel, sp… Show more

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Cited by 31 publications
(29 citation statements)
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References 186 publications
(194 reference statements)
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“…Existing studies have determined that gene expression in PAH is also regulated by epigenetic factors, including DNA methylation, interference of microRNAs and histone modification, but these processes do not change the sequence of genes [ 30 , 31 ]. In 2010, Archer et al’s [ 32 ] investigation first demonstrated that epigenetic deficiency of superoxide dismutase (SOD)-2 due to gene methylation in an enhancer region of intron 2 and in the promoter triggered abnormal proliferation and apoptosis resistance in a heritable PAH rat model.…”
Section: Pathogenesis Of Pahmentioning
confidence: 99%
“…Existing studies have determined that gene expression in PAH is also regulated by epigenetic factors, including DNA methylation, interference of microRNAs and histone modification, but these processes do not change the sequence of genes [ 30 , 31 ]. In 2010, Archer et al’s [ 32 ] investigation first demonstrated that epigenetic deficiency of superoxide dismutase (SOD)-2 due to gene methylation in an enhancer region of intron 2 and in the promoter triggered abnormal proliferation and apoptosis resistance in a heritable PAH rat model.…”
Section: Pathogenesis Of Pahmentioning
confidence: 99%
“…PAH was initially described as a non-treatable disease. However, the recent advances in diagnostic tools, multi-omics technologies, and early treatment have significantly improved patients’ quality of life and overall life expectancy [ 15 , 16 , 17 ]. Current data shows that the median survival was 2.8 years between 1980 and 1991 when no specific and efficient treatments were available, while the median survival is 7–10 years in the modern treatment era [ 18 , 19 ].…”
Section: Current Therapies In Pahmentioning
confidence: 99%
“…Over the past decade, accumulated data suggested that epigenetics may play a major role in the setting of PAH ( Bisserier et al, 2020 ; Cheng et al, 2019 ). Epigenetics is defined as a heritable change to the chromatin resulting in a shift in gene expression without altering the DNA sequence ( Weinhold, 2006 ).…”
Section: Emerging Therapiesmentioning
confidence: 99%
“…Pathophysiologic characteristics of PH include vasoconstriction, thrombosis and inflammation. Pulmonary vascular remodeling is associated with smooth muscle and endothelial cells dysfunction ( Bisserier et al, 2020 ; Shimoda and Laurie, 2013 ). It is now well established that pulmonary artery endothelial cells (PAECs) play a major role in the regulation of vascular tone, inflammation, and thrombosis.…”
Section: Introductionmentioning
confidence: 99%