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• W2792825729 abstract "Despite the discovery more than 15 years ago that patients with hereditary pulmonary arterial hypertension (HPAH) inherit BMP type 2 receptor ( BMPR2) mutations, it is still unclear how these mutations cause disease. In part, this is attributable to the rarity of HPAH and difficulty obtaining tissue samples from patients with early disease. However, in addition, limitations to the approaches used to study the effects of BMPR2 mutations on the pulmonary vasculature have restricted our ability to determine how individual mutations give rise to progressive pulmonary vascular pathology in HPAH. The importance of understanding the mechanisms by which BMPR2 mutations cause disease in patients with HPAH is underscored by evidence that there is reduced BMPR2 expression in patients with other, more common, non-hereditary form of PAH, and that restoration of BMPR2 expression reverses established disease in experimental models of pulmonary hypertension. In this paper, we focus on the effects on endothelial function. We discuss some of the controversies and challenges that have faced investigators exploring the role of BMPR2 mutations in HPAH, focusing specifically on the effects different BMPR2 mutation have on endothelial function, and whether there are qualitative differences between different BMPR2 mutations. We discuss evidence that BMPR2 signaling regulates a number of responses that may account for endothelial abnormalities in HPAH and summarize limitations of the models that are used to study these effects. Finally, we discuss evidence that BMPR2-dependent effects on endothelial metabolism provides a unifying explanation for the many of the BMPR2 mutation-dependent effects that have been described in patients with HPAH." @default.
• W2792825729 created "2018-03-29" @default.
• W2792825729 creator A5009531733 @default.
• W2792825729 creator A5049719553 @default.
• W2792825729 creator A5063079256 @default.
• W2792825729 date "2018-04-01" @default.
• W2792825729 modified "2023-10-13" @default.
• W2792825729 title "<i>BMPR2</i> mutations and endothelial dysfunction in pulmonary arterial hypertension (2017 Grover Conference Series)" @default.
• W2792825729 cites W1533684438 @default.
• W2792825729 cites W1606441445 @default.
• W2792825729 cites W1824340961 @default.
• W2792825729 cites W1899124983 @default.
• W2792825729 cites W1932302589 @default.
• W2792825729 cites W1964902913 @default.
• W2792825729 cites W1965971393 @default.
• W2792825729 cites W1966971152 @default.
• W2792825729 cites W196831495 @default.
• W2792825729 cites W1986081466 @default.
• W2792825729 cites W1994324715 @default.
• W2792825729 cites W1996248643 @default.
• W2792825729 cites W1997547080 @default.
• W2792825729 cites W1997617706 @default.
• W2792825729 cites W1999275474 @default.
• W2792825729 cites W2001098033 @default.
• W2792825729 cites W2001510319 @default.
• W2792825729 cites W2003155673 @default.
• W2792825729 cites W2012675069 @default.
• W2792825729 cites W2014751423 @default.
• W2792825729 cites W2020646157 @default.
• W2792825729 cites W2028827931 @default.
• W2792825729 cites W2030470655 @default.
• W2792825729 cites W2035005764 @default.
• W2792825729 cites W2037568417 @default.
• W2792825729 cites W2041507819 @default.
• W2792825729 cites W2046087773 @default.
• W2792825729 cites W2046658218 @default.
• W2792825729 cites W2049404716 @default.
• W2792825729 cites W2051524933 @default.
• W2792825729 cites W2054084651 @default.
• W2792825729 cites W2056356945 @default.
• W2792825729 cites W2070004323 @default.
• W2792825729 cites W2074858413 @default.
• W2792825729 cites W2077065151 @default.
• W2792825729 cites W2080653159 @default.
• W2792825729 cites W2087714835 @default.
• W2792825729 cites W2088465910 @default.
• W2792825729 cites W2089273856 @default.
• W2792825729 cites W2089812396 @default.
• W2792825729 cites W2090798565 @default.
• W2792825729 cites W2095987251 @default.
• W2792825729 cites W2096393477 @default.
• W2792825729 cites W2099912088 @default.
• W2792825729 cites W2104874723 @default.
• W2792825729 cites W2105891302 @default.
• W2792825729 cites W2107763047 @default.
• W2792825729 cites W2107821473 @default.
• W2792825729 cites W2110051509 @default.
• W2792825729 cites W2117417680 @default.
• W2792825729 cites W2119521027 @default.
• W2792825729 cites W2123354521 @default.
• W2792825729 cites W2124495107 @default.
• W2792825729 cites W2128940917 @default.
• W2792825729 cites W2133993493 @default.
• W2792825729 cites W2134915108 @default.
• W2792825729 cites W2136364191 @default.
• W2792825729 cites W2139960175 @default.
• W2792825729 cites W2146234408 @default.
• W2792825729 cites W2146730628 @default.
• W2792825729 cites W2147296845 @default.
• W2792825729 cites W2148965526 @default.
• W2792825729 cites W2149480398 @default.
• W2792825729 cites W2157122261 @default.
• W2792825729 cites W2158364013 @default.
• W2792825729 cites W2162865304 @default.
• W2792825729 cites W2163394057 @default.
• W2792825729 cites W2167099419 @default.
• W2792825729 cites W2171273257 @default.
• W2792825729 cites W2236397223 @default.
• W2792825729 cites W2253353716 @default.
• W2792825729 cites W2326299596 @default.
• W2792825729 cites W2332627679 @default.
• W2792825729 cites W2338679546 @default.
• W2792825729 cites W2341828371 @default.
• W2792825729 cites W2404128784 @default.
• W2792825729 cites W2510865896 @default.
• W2792825729 cites W2518252291 @default.
• W2792825729 cites W2532286205 @default.
• W2792825729 cites W2555492566 @default.
• W2792825729 cites W2592714610 @default.
• W2792825729 cites W2593784471 @default.
• W2792825729 cites W2609733517 @default.
• W2792825729 cites W2617937955 @default.
• W2792825729 cites W2636866226 @default.
• W2792825729 cites W2740127513 @default.
• W2792825729 cites W2740191821 @default.
• W2792825729 doi "https://doi.org/10.1177/2045894018765840" @default.
• W2792825729 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/5912278" @default.
• W2792825729 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/29521190" @default.

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