Selected Basic Scientific Papers on PAH 2017 1. Allawzi AM, Vang A, Clements RT, Jhun BS, Kue NR, Mancini TJ, Landi AK, Terentyev D, O-Uchi J, Comhair SA, Erzurum SC, Choudhary G. (in press). Activation of Anoctamin-1 Limits Pulmonary Endothelial Cell Proliferation via p38-MAPK-dependent Apoptosis. American Journal of Respiratory Cell and Molecular Biology. 2017. https://doi.org/10.1165/rcmb.2016-0344OC. 2. Asosingh K, Wanner N, Weiss K, Queisser K, Gebreab L, Kassa B, Stuehr E, Graham B, Erzurum S. Bone marrow transplantation prevents right ventricle disease in the caveolin-1– deficient mouse model of pulmonary hypertension. Blood Advances . 2017; 1(9):526-534. https://doi.org/10.1182/bloodadvances.2016002691 3. Mous DS, Buscop-van Kempen MJ, Wijnen RMH, Tibboel D, Rottier RJ. Changes in vasoactive pathways in congenital diaphragmatic hernia associated pulmonary hypertension explain unresponsiveness to pharmacotherapy. Respiratory Research . 2017; 18:187. https://doi.org/10.1186/s12931-017-0670-2 4. Tojais NF, Cao A, Lai YJ, Wang L, Chen PI, Alejandre Alcazar MA, de Jesus Perez VA, Hopper RK, Rhodes CJ, Bill MA, Sakai LY, Rabinovitch M. Codependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor- β in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension. Arteriosclerosis, Thrombosis, and Vascular Biology . 2017; 37:1559-1569. http://atvb.ahajournals.org/content/37/8/1559 5. Prins KW, Tian L, Wu D, Thenappan T, Metzger JM, Archer SL. Colchicine Depolymerizes Microtubules, Increases Junctophilin - 2, and Improves Right Ventricular Function in Experimental Pulmonary Arterial Hypertension. Journal of the American Heart Association . 2017; 6:e006195. http://jaha.ahajournals.org/content/6/6/e006195 6. Bai Y, Li ZX, Zhao YT, Liu M, Wang Y, Lian GC, Zhao Q, Wang HL. PCPA protects against monocrotaline-induced pulmonary arterial remodeling in rats: potential roles of connective tissue growth factor. Oncotarget . 2017; 8(67):111642-111655. https://doi.org/10.18632/oncotarget.22882 7. Truong QA, Bhatia HS, Szymonifka J, Zhou Q, Lavender Z, Waxman AB, Semigran MJ, Malhotra R. A four-tier classification system of pulmonary artery metrics on computed tomography for the diagnosis and prognosis of pulmonary hypertension. Journal of Cardiovascular Computed Tomography . 2018; 12:60-66. http://www.journalofcardiovascularct.com/article/S1934-5925(17)30259-9/fulltext 8. Chopard R, Genet B, Ecarnot F, Chatot M, Napporn G, Hyvert A, Didier-Petit K, Schiele F, Meneveau N. Detection of Residual Pulmonary Vascular Obstruction by Ventilation- Perfusion Lung Scan Late After a First Pulmonary Embolism. The American Journal of Cardiology . 2017; 119(11):1883-1889. https://doi.org/10.1016/j.amjcard.2017.03.002
9. Hautefort A, Chesné J, Preussner J, Pullamsetti SS, Tost J, Looso M, Antigny F, Girerd B, Riou M, Eddahibi S, Deleuze JF, Seeger W, Fade E, Simonneau G, Montani D, Humbert M, Perros F. Pulmonary endothelial cell DNA methylation signature in pulmonary arterial hypertension. Oncotarget . 2017; 8(32):52995-53016. https://doi.org/10.18632/oncotarget.18031 10. Tavares-Silva M, Alaa M, Leite S, Oliveira-Pinto J, Lopes L, Leite-Moreira AF, Lourenço AP. Dose-Response Head-to-Head Comparison of Inodilators Dobutamine, Milrinone, and Levosimendan in Chronic Experimental Pulmonary Hypertension. Journal of Cardiovascular Pharmacology and Therapeutics . 2017; 22(5):485-495. https://doi.org/10.1177/1074248417696818 11. Zhu B, Gong Y, Yan G, Wang D, Qiao Y, Wang Q, Liu B, Hou J, Li R, Tang C. Down- regulation of lncRNA MEG3 promotes hypoxia-induced human pulmonary artery smooth muscle cell proliferation and migration via repressing PTEN by sponging miR-21. Biochemical and Biophysical Research Communications. 2017; 495(3):2125-2132. https://www.sciencedirect.com/science/article/pii/S0006291X17323628 12. Egnatchik RA, Brittain EL, Shah AT, Fares WH, Ford HJ, Monahan K, Kang CJ, Kocurek EG, Zhu S, Luong T, Nguyen TT, Hysinger E, Austin ED, Skala MC, Young JD, Roberts LJ II, Hemnes AR, West J, Fesse JP. Dysfunctional BMPR2 signaling drives an abnormal endothelial requirement for glutamine in pulmonary arterial hypertension. Pulmonary Circulation . 2017; 7(1):186-199. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448547/# 13. Hadinnapola C, Bleda M, Haimel M, Screaton N, Swift A, Dorfmüller P, Preston SD, Southwood M, Hernandez-Sanchez J, Martin J, Treacy C, Yates K, Bogaard H, Church C, Coghlan G, Condliffe R, Corris PA, Gibbs S, Girerd B, Holden S, Humbert M, Kiely DG, Lawrie A, Machado R, MacKenzie Ross R, Moledina S, Montani D, NewnhamM, Peacock A, Pepke-Zaba J, Rayner-Matthews P, Shamardina O, Soubrier F, Southgate L, Suntharalingam J, Toshner M, Trembath R, Vonk Noordegraaf A, Wilkins MR, Wort SJ, Wharton J, Gräf S, Morrell NW. Phenotypic Characterization of EIF2AK4 Mutation Carriers in a Large Cohort of Patients Diagnosed Clinically With Pulmonary Arterial Hypertension. Circulation . 2017; 136:2022-2033. https://doi.org/10.1161/CIRCULATIONAHA.117.028351 14. Roan JN, Hsu CH, Fang SY, Tsai HW, Luo CY, Huang CC, Lam CF. Exendin-4 improves cardiovascular function and survival in flow-induced pulmonary hypertension. The Journal of Thoracic and Cardiovascular Surgery . 2018; 155(4):1661-1669. http://www.jtcvsonline.org/article/S0022-5223(17)32432-7/fulltext 15. Saygin D, Highland KB, Farha S, Park M, Sharp J, Roach EC, Wilson Tang WH, Thomas JD, Erzurum SC, Neumann DR, DiFilippo FP. Metabolic and Functional Evaluation of the Heart and Lungs in Pulmonary Hypertension by Gated 2-[18F]-Fluoro-2-deoxy-D-glucose Positron Emission Tomography. Pulmonary Circulation . 2017; 7(2):428-438. https://doi.org/10.1177/2045893217701917
16. Bourgeois A, Lambert C, Habbout K, Ranchoux B, Paquet-Marceau S, Trinh I, Breuils- Bonnet S, Paradis R, Nadeau V, Paulin R, Provencher S, Bonnet S, Boucherat O. FOXM1 promotes pulmonary artery smooth muscle cell expansion in pulmonary arterial hypertension. Journal of Molecular Medicine . 2018; 96(2):223-235. https://doi.org/10.1007/s00109-017-1619-0 17. Hemnes AR, Humbert M. Pathobiology of pulmonary arterial hypertension: understanding the roads less travelled. European Respiratory Review . 2017; 26:170093. https://doi.org/10.1183/16000617.0093-2017 18. Caruso P, Dunmore BJ, Schlosser K, Schoors S, Dos Santos C, Perez-Iratxeta C, Lavoie JR, Zhang H, Long L, Flockton AR, Frid MG, Upton PD, D’Alessandro A, Hadinnapola C, Kiskin FN, Taha M, Hurst LA, Ormiston ML, Hata A, Stenmark KR, Carmeliet P, Stewart DJ, Morrell NW. Identification of MicroRNA-124 as a Major Regulator of Enhanced Endothelial Cell Glycolysis in Pulmonary Arterial Hypertension via PTBP1 (Polypyrimidine Tract Binding Protein) and Pyruvate Kinase M2. Circulation . 2017; 136:2451-2467. https://doi.org/10.1161/CIRCULATIONAHA.117.028034 19. Takagi K, Yamakuchi M, Matsuyama T, Kondo K, Uchida A, Misono S, Hashiguchi T, Inoue H. IL-13 enhances mesenchymal transition of pulmonary artery endothelial cells via down-regulation of miR-424/503 in vitro. Cellular Signalling . 2018; 42:270-280. https://doi.org/10.1016/j.cellsig.2017.10.019 20. Michelakis ED, Gurtu V, Webster L, Barnes G, Watson G, Howard L, Cupitt J, Paterson I, Thompson RB, Chow K, O’Regan DP, Zhao L, Wharton J, Kiely DG, Kinnaird A, Boukouris AE, White C, Nagendran J, Freed DH, Wort SJ, Gibbs JSR, Wilkins MR. Inhibition of pyruvate dehydrogenase kinase improves pulmonary arterial hypertension in genetically susceptible patients. Science Translational Medicine . 2017; 9(413):eaao4583. http://stm.sciencemag.org/content/9/413/eaao4583 21. Sala MA, Chen C, Zhang Q, Do-Umehara HC, Wu W, Misharin AV, Waypa GB, Fang D, Budinger GRS, Liu S, Chandel NS, Schumacker PT, Sznajder JI, Liu J. JNK2 up-regulates hypoxia-inducible factors and contributes to hypoxia-induced erythropoiesis and pulmonary hypertension. Journal of Biological Chemistry . 2018; 293(1):271-284. http://www.jbc.org/content/293/1/271 22. Fu LC, Lv Y, Zhong Y, He Q, Liu X, Du LZ. Tyrosine phosphorylation of Kv1.5 is upregulated in intrauterine growth retardation rats with exaggerated pulmonary hypertension. Brazilian Journal of Medical and Biological Research . 2017; 50(11):e6237. http://dx.doi.org/10.1590/1414-431x20176237
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