Selected Basic Scientific Papers on PAH 2017 1. Allawzi AM, Vang A, - - PDF document

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Selected Basic Scientific Papers on PAH 2017 1. Allawzi AM, Vang A, - - PDF document

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


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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
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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

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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

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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

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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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23. Shen C, Romero M, Brunelle A, Wolfe C, Dobyns A, Francis M, Taylor MS, Puglisi J, Longo LD, Zhang L, Wilson CG, Wilson SM. Long-Term High Altitude Hypoxia influences Pulmonary Arterial L-type Calcium Channel mediated Ca2+ signals and Contraction in Fetal and Adult Sheep. American Journal of Physiology Regulatory, Integrative and Comparative Physiology. 2018; 314(3):R433-R446. https://www.physiology.org/doi/10.1152/ajpregu.00154.2017 24. Marsboom G, Chen Z, Yuan Y, Zhang Y, Tiruppathi C, Loyd JE, Austin ED, Machado RF, Minshall RD, Rehman J, Malik AB. Aberrant caveolin-1–mediated Smad signaling and proliferation identified by analysis of adenine 474 deletion mutation (c.474delA) in patient fibroblasts: a new perspective on the mechanism of pulmonary hypertension. Molecular Biology of the Cell. 2017; 28(9) :1177-1185. https://www.molbiolcell.org/doi/10.1091/mbc.e16-11-0790 25. Hong Z, Chen KH, DasGupta A, Potus F, Dunham-Snary K, Bonnet S, Tian L, Fu J, Breuils-Bonnet S, Provencher S, Wu D, Mewburn J, Ormiston ML, Archer SL. MicroRNA-138 and MicroRNA-25 Down-regulate Mitochondrial Calcium Uniporter, Causing the Pulmonary Arterial Hypertension Cancer Phenotype. American Journal of Respiratory and Critical Care Medicine. 2017; 195(4):515-529. https://www.atsjournals.org/doi/full/10.1164/rccm.201604-0814OC 26. Zhang H, Wang D, Li M, Plecitá-Hlavatá L, D’Alessandro A, Tauber J, Riddle S, Kumar S, Flockton A, McKeon BA, Frid MG, Reisz JA, Caruso P, El Kasmi KC, Ježek P, Morrell NW, Hu CJ, Stenmark KR. Metabolic and Proliferative State of Vascular Adventitial Fibroblasts in Pulmonary Hypertension Is Regulated Through a MicroRNA-124/PTBP1 (Polypyrimidine Tract Binding Protein 1)/Pyruvate Kinase Muscle Axis. Circulation. 2017; 136:2468-2485. http://circ.ahajournals.org/content/136/25/2468 27. Rhodes CJ, Ghataorhe P, Wharton J, Rue-Albrecht KC, Hadinnapola C, Watson G, Bleda M, Haimel M, Coghlan G, Corris PA, Howard LS, Kiely DG, Peacock AJ, Pepke-Zaba J, Toshner MR, Wort SJ, Gibbs JSR, Lawrie A, Gräf S, Morrell NW, Wilkins MR. Plasma Metabolomics Implicates Modified Transfer RNAs and Altered Bioenergetics in the Outcomes of Pulmonary Arterial Hypertension. Circulation. 2017; 135:460-475. http://circ.ahajournals.org/content/135/5/460 28. Sysol JR, Chen J, Singla S, Zhao S, Comhair S, Natarajan V, Machado RF. Micro-RNA-1 is decreased by hypoxia and contributes to the development of pulmonary vascular remodeling via regulation of sphingosine kinase 1. American Journal of Physiology Lung Cellular and Molecular Physiology. 2018; 314(3):L461-L472. https://www.physiology.org/doi/10.1152/ajplung.00057.2017 29.

  • O. Kheyfets V, Sucharov CC, Truong U, Dunning J, Hunter K, Ivy D, Miyamoto S,

Shandas R. Circulating miRNAs in Pediatric Pulmonary Hypertension Show Promise as Biomarkers of Vascular Function. Oxidative Medicine and Cellular Longevity. 2017. https://www.hindawi.com/journals/omcl/2017/4957147/

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30. Goddard JC, Armstrong IJ, Kiely DG, Elliot CA, Charalampopoulos A, Condliffe R, Stone BJ, Sabroe I. Combining creative writing and narrative analysis to deliver new insights into the impact of pulmonary hypertension. BMJ Open Respiratory Research. 2017; 4(1):e000184. http://bmjopenrespres.bmj.com/content/4/1/e000184.info 31. Xu S, Xu X, Zhang J, Ying K, Shao Y, Zhang R. Pulmonary hypertension as a manifestation of mitochondrial disease: A case report and review of the literature.

  • Medicine. 2017; 96(46):e8716. http://dx.doi.org/10.1097/MD.0000000000008716

32. McCulley DJ, Wienhold MD, Hines EA, Hacker TA, Rogers A, Pewowaruk RJ, Zewdu R, Chesler NC, Selleri L, Sun X. PBX transcription factors drive pulmonary vascular adaptation to birth. The Journal of Clinical Investigation. 2018; 128(2):655-667. https://www.jci.org/articles/view/93395 33. Kimura M, Tamura Y, Guignabert C, Takei M, Kosaki K, Tanabe N, Tatsumi K, Saji T, Satoh T, Kataoka M, Kamitsuji S, Kamatani N, Thuillet R, Tu L, Humbert M, Fukuda K, Sano M. A genome-wide association analysis identifies PDE1A|DNAJC10 locus on chromosome 2 associated with idiopathic pulmonary arterial hypertension in a Japanese

  • population. Oncotarget. 2017; 8(43):74917-74926.

https://doi.org/10.18632/oncotarget.20459 34. Badr Eslam R, Croce K, Marinho Mangione F, Musmann R, Leopold JA, Mitchell RN, Waxman AB. Persistence and proliferation of human mesenchymal stromal cells in the right ventricular myocardium after intracoronary injection in a large animal model of pulmonary hypertension. Cytotherapy - The Journal of Cell Therapy. 2017; 19(6):668-679. http://www.celltherapyjournal.org/article/S1465-3249(17)30426-7/fulltext 35. Parikh R, Varghese B, Khatoon HN, Kovach JA, Kavinsky CJ, Tandon R. Increased mortality from complications of pulmonary hypertension in patients undergoing transcatheter aortic valve replacement. Pulmonary Circulation. 2017; 7(2):391-398. http://journals.sagepub.com/doi/full/10.1177/2045893217697709 36. Pena A, Kobir A, Goncharov D, Goda A, Kudryashova TV, Ray A, Vanderpool R, Baust J, Chang B, Mora AL, Gorcsan J III, Goncharova EA. Pharmacological Inhibition of mTOR Kinase Reverses Right Ventricle Remodeling and Improves Right Ventricle Structure and Function in Rats. American Journal of Respiratory Cell and Molecular Biology. 2017; 57(5):615-625. https://www.atsjournals.org/doi/full/10.1165/rcmb.2016-0364OC 37. Nguyen QL, Corey C, White P, Watson A, Gladwin MT, Simon MA, Shiv S. Platelets from pulmonary hypertension patients show increased mitochondrial reserve capacity. JCI

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38. Xu Y, Gu Q, Liu N, Yan Y, Yang X, Hao Y, Qu C. PPARγ Alleviates Right Ventricular Failure Secondary to Pulmonary Arterial Hypertension in Rats. International Heart

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39. Nossent EJ, Antigny F, Montani D, Bogaard HJ, Ghigna MR, Lambert M, de Montpréville VT, Girerd B, Jaïs X, Savale L, Mercier O, Fadel E, Soubrier F, Sitbon O, Simonneau G, Vonk Noordegraaf A, Humbert M, Perros F, Dorfmüller P. (in press). Pulmonary vascular remodeling patterns and expression of general control nonderepressible 2 (GCN2) in pulmonary veno-occlusive disease. The Journal of Heart and Lung Transplantation. 2017. http://www.jhltonline.org/article/S1053-2498(17)32037-5/fulltext 40. Maloney JP. Putting skin in the game: dermis-derived stem cells provide insight into familial pulmonary hypertension. Stem Cell Investigation. 2017; 4:35. http://sci.amegroups.com/article/view/14724/14909 41. Hemnes AR, Beck GJ, Newman JH, Abidov A, Aldred MA, Barnard J, Berman Rosenzweig E, Borlaug BA, Chung WK, Comhair SAA, Erzurum SC, Frantz RP, Gray MP, Grunig G, Hassoun PM, Hill NS, Horn EM, Hu B, Lempel JK, Maron BA, Mathai SC, Olman MA, Rischard FP, Systrom DM, Wilson Tang WH, Waxman AB, Xiao L, Yuan JXJ, Leopold JA. PVDOMICS A Multi-Center Study to Improve Understanding of Pulmonary Vascular Disease Through Phenomics. Circulation Research. 2017; 121(10):1136-1139. http://circres.ahajournals.org/content/121/10/1136 42. Gu M, Shao NY, Sa S, Li D, Termglinchan V, Ameen M, Karakikes I, Sosa G, Grubert F, Lee J, Cao A, Taylor S, Ma Y, Zhao Z, Chappell J, Hamid R, Austin ED, Gold JD, Wu JC, Snyder MP, Rabinovitch M. Patient-Specific iPSC-Derived Endothelial Cells Uncover Pathways that Protect against Pulmonary Hypertension in BMPR2 Mutation Carriers. Cell Stem Cell. 2017; 20(4):490-504. http://www.cell.com/cell-stem-cell/fulltext/S1934- 5909(16)30266-1 43. Neto-Neves EM, Frump AL, Vayl A, Kline JA, Lahm T. Isolated heart model demonstrates evidence of contractile and diastolic dysfunction in right ventricles from rats with sugen/hypoxia-induced pulmonary hypertension. Physiological Reports. 2017; 5(19):e13438. https://physoc.onlinelibrary.wiley.com/doi/abs/10.14814/phy2.13438 44. Sankhe S, Manousakidi S, Antigny F, Arthur Ataam J, Bentebbal S, Ruchon Y, Lecerf F, Sabourin J, Price L, Fadel E, Dorfmüller P, Eddahibi S, Humbert M, Perros F, Capuano V. T-type Ca2 + channels elicit pro-proliferative and anti-apoptotic responses through impaired PP2A/Akt1 signaling in PASMCs from patients with pulmonary arterial hypertension. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research. 2017; 1864(10): 1631-

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46. Saito T, Miyagawa K, Chen SY, Tamosiuniene R, Wang L, Sharpe O, Samayoa E, Harada D, Moonen JRAJ, Cao A, Chen PI, Hennigs JK, Gu M, Li CG, Leib RD, Li D, Adams CM, del Rosario PA, Bill M, Haddad F, Montoya JG, Robinson WH, Fantl WJ, Nolan GP, Zamanian RT, Nicolls MR, Chiu CY, Ariza ME, Rabinovitch M. Upregulation of Human Endogenous Retrovirus-K Is Linked to Immunity and Inflammation in Pulmonary Arterial

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