Li W, Long L, Yang X, Tong Z, Southwood M, King R, Caruso P, Upton PD, Yang P, Bocobo GA, Nikolic I, Higuera A, Salmon RM, Jiang H, Lodge KM, Hoenderdos K, Baron RM, Yu PB, Condliffe AM, Summers C, Nourshargh S, Chilvers ER, Morrell NW. Circulating BMP9 Protects the Pulmonary Endothelium During Inflammation-induced Lung Injury in Mice. Am J Respir Crit Care Med. 2020 Dec 15. doi: 10.1164/rccm.202005-1761OC. Online ahead of print. PMID: 33320799
Swietlik EM, Greene D, Zhu N, Megy K, Cogliano M, Rajaram S, Pandya D, Tilly T, Lutz KA, Welch CCL, Pauciulo MW, Southgate L, Martin JM, Treacy CM, Penkett CJ, Stephens JC, Bogaard HJ, Church C, Coghlan G, Coleman AW, Condliffe R, Eichstaedt CA, Eyries M, Gall H, Ghio S, Girerd B, Grünig E, Holden S, Howard L, Humbert M, Kiely DG, Kovacs G, Lordan J, Machado RD, Mackenzie Ross RV, McCabe C, Moledina S, Montani D, Olschewski H, Pepke-Zaba J, Price L, Rhodes CJ, Seeger W, Soubrier F, Suntharalingam J, Toshner MR, Vonk Noordegraaf A, Wharton J, Wild JM, Wort SJ, Lawrie A, Wilkins MR, Trembath RC, Shen Y, Chung WK, Swift AJ, Nichols WC, Morrell NW*, Gräf S* .Bayesian Inference Associates Rare KDR Variants with Specific Phenotypes in Pulmonary Arterial Hypertension. Circ Genom Precis Med. 2020 Dec 15. doi: 10.1161/CIRCGEN.120.003155. Online ahead of print. * joint senior author PMID: 33320693
Emanuelli G, Nassehzadeh-Tabriz N, Morrell NW, Marciniak SJ. The integrated stress response in pulmonary disease. Eur Respir Rev. 2020 Oct 1;29(157):200184. PMID: 33004527
Long L, Yang X, Southwood M, Moore S, Crosby A, Upton PD, Dunmore BJ, Morrell NW. Targeting translational read-through of premature termination mutations in BMPR2 with PTC124 for pulmonary arterial hypertension. Pulm Circ. 2020;10(3):2045894020935783. PMID: 32733669
Salmon RM, Guo J, Wood JH, Tong Z, Beech JS, Lawera A, Yu M, Grainger DJ, Reckless J,Morrell NW, Li W. Molecular basis of ALK1-mediated signalling by BMP9/BMP10 and their prodomain-bound forms. Nat Commun. 2020 Apr 1;11(1):1621. PMID: 32238803
Dunmore BJ, Yang X, Crosby A, Moore S, Long L, Huang C, Southwood M, Austin ED, Rana A, Upton PD, Morrell NW. 4PBA Restores Signaling of a Cysteine-substituted Mutant BMPR2 Receptor Found in Patients with Pulmonary Arterial Hypertension. Am J Respir Cell Mol Biol.2020 Aug;63(2):160-171. PMID: 32255665
Owen NE, Alexander GJ, Sen S, Bunclark K, Polwarth G, Pepke-Zaba J, Davenport AP, Morrell NW, Upton PD.Reduced circulating BMP10 and BMP9 and elevated endoglin are associated with disease severity, decompensation and pulmonary vascular syndromes in patients with cirrhosis. EBioMedicine. 2020 Jun;56:102794. PMID: 32454407
2019
****Hodgson J, Swietlik EM, Salmon RM, Hadinnapola C, Nikolic I, Wharton J, Guo J, Liley J, Haimel M, Bleda M, Southgate L, Machado RD, Martin JM, Treacy CM, Yates K, Daugherty LC, Shamardina O, Whitehorn D, Holden S, Bogaard HJ, Church C, Coghlan G, Condliffe R, Corris PA, Danesino C, Eyries M, Gall H, Ghio S, Ghofrani HA, Gibbs JSR, Girerd B, Houweling AC, Howard L, Humbert M, Kiely DG, Kovacs G, Lawrie A, MacKenzie Ross RV, Moledina S, Montani D, Olschewski A, Olschewski H, Ouwehand WH, Peacock AJ, Pepke-Zaba J, Prokopenko I, Rhodes CJ, Scelsi L, Seeger W, Soubrier F, Suntharalingam J, Toshner MR, Trembath RC, Vonk Noordegraaf A, Wort SJ, Wilkins MR, Yu PB, Li W, Gräf S, Upton PD, Morrell NW. Characterization of GDF2 Mutations and Levels of BMP9 and BMP10 in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2019 Oct 29. doi: 10.1164/rccm.201906-1141OC. PMID: 31661308
Tong Z, Guo J, Glen RC,Morrell NW, Li W. A Bone Morphogenetic Protein (BMP)-derived Peptide Based on the Type I Receptor-binding Site Modifies Cell-type Dependent BMP Signalling. Sci Rep. 2019 Sep 17;9(1):13446. PMID: 31530856
Lawera A, Tong Z, Thorikay M, Redgrave RE, Cai J, van Dinther M,Morrell NW, Afink GB, Charnock-Jones DS, Arthur HM, Ten Dijke P, Li W.Role of soluble endoglin in BMP9 signaling. Proc Natl Acad Sci U S A. 2019 Sep 3;116(36):17800-17808. PMID: 31431534
Southgate L, Machado RD, Gräf S,Morrell NW. Molecular genetic framework underlying pulmonary arterial hypertension. Nat Rev Cardiol. 2019 Aug 12. doi: 10.1038/s41569-019-0242-x. PMID: 31406341
Gomez-Puerto MC, van Zuijen I, Huang CJ, Szulcek R, Pan X, van Dinther MA, Kurakula K, Wiesmeijer CC, Goumans MJ, Bogaard HJ,Morrell NW, Rana AA, Ten Dijke P. Autophagy contributes to BMP type 2 receptor degradation and development of pulmonary arterial hypertension. J Pathol. 2019 Nov;249(3):356-367. PMID: 31257577
Sofianopoulou E, Kaptoge S, Gräf S, Hadinnapola C, Treacy CM, Church C, Coghlan G, Gibbs JSR, Haimel M, Howard LS, Johnson M, Kiely DG, Lawrie A, Lordan J, MacKenzie Ross RV, Martin JM, Moledina S, Newnham M, Peacock AJ, Price LC, Rhodes CJ, Suntharalingam J, Swietlik EM, Toshner MR, Wharton J, Wilkins MR, Wort SJ, Pepke-Zaba J, Condliffe R, Corris PA, Di Angelantonio E, Provencher S,Morrell NW. Traffic exposures, air pollution and outcomes in pulmonary arterial hypertension: a UK cohort study analysis. Eur Respir J. 2019 May 30;53(5). pii: 1801429. PMID: 30923185
***Rhodes CJ, Batai K, Bleda M, Haimel M, Southgate L, Germain M, Pauciulo MW, Hadinnapola C, Aman J, Girerd B, Arora A, Knight J, Hanscombe KB, Karnes JH, Kaakinen M, Gall H, Ulrich A, Harbaum L, Cebola I, Ferrer J, Lutz K, Swietlik EM, Ahmad F, Amouyel P, Archer SL, Argula R, Austin ED, Badesch D, Bakshi S, Barnett C, Benza R, Bhatt N, Bogaard HJ, Burger CD, Chakinala M, Church C, Coghlan JG, Condliffe R, Corris PA, Danesino C, Debette S, Elliott CG, Elwing J, Eyries M, Fortin T, Franke A, Frantz RP, Frost A, Garcia JGN, Ghio S, Ghofrani HA, Gibbs JSR, Harley J, He H, Hill NS, Hirsch R, Houweling AC, Howard LS, Ivy D, Kiely DG, Klinger J, Kovacs G, Lahm T, Laudes M, Machado RD, MacKenzie Ross RV, Marsolo K, Martin LJ, Moledina S, Montani D, Nathan SD, Newnham M, Olschewski A, Olschewski H, Oudiz RJ, Ouwehand WH, Peacock AJ, Pepke-Zaba J, Rehman Z, Robbins I, Roden DM, Rosenzweig EB, Saydain G, Scelsi L, Schilz R, Seeger W, Shaffer CM, Simms RW, Simon M, Sitbon O, Suntharalingam J, Tang H, Tchourbanov AY, Thenappan T, Torres F, Toshner MR, Treacy CM, Vonk Noordegraaf A, Waisfisz Q, Walsworth AK, Walter RE, Wharton J, White RJ, Wilt J, Wort SJ, Yung D, Lawrie A, Humbert M, Soubrier F, Trégouët DA, Prokopenko I, Kittles R, Gräf S, Nichols WC, Trembath RC, Desai AA,Morrell NW, Wilkins MR; UK NIHR BioResource Rare Diseases Consortium; UK PAH Cohort Study Consortium; US PAH Biobank Consortium. Genetic determinants of risk in pulmonary arterial hypertension: international genome-wide association studies and meta-analysis. Lancet Respir Med. 2019 Mar;7(3):227-238. (joint senior and corresponding author). PMID: 30527956
2018
Ferrer E, Dunmore BJ, Hassan D, Ormiston ML, Moore S, Deighton J, Long L, Yang XD, Stewart DJ, Morrell NW. A potential role for exosomal TCTP export in vascular remodellingin pulmonary arterial hypertension. Am J Respir Cell Mol Biol. 2018;59(4):467-478 PMID: 29676587
*** Gräf S, Haimel M, Bleda M, Hadinnapola C, Southgate L, Li W, Hodgson J, Liu B, Salmon RM, Southwood M, Machado RD, Martin JM, Treacy CM, Yates K, Daugherty LC, Shamardina O, Whitehorn D, Holden S, Aldred M, Bogaard HJ, Church C, Coghlan G, Condliffe R, Corris PA, Danesino C, Eyries M, Gall H, Ghio S, Ghofrani HA, Gibbs JSR, Girerd B, Houweling AC, Howard L, Humbert M, Kiely DG, Kovacs G, MacKenzie Ross RV, Moledina S, Montani D, Newnham M, Olschewski A, Olschewski H, Peacock AJ, Pepke-Zaba J, Prokopenko I, Rhodes CJ, Scelsi L, Seeger W, Soubrier F, Stein DF, Suntharalingam J, Swietlik EM, Toshner MR, van Heel DA, Vonk Noordegraaf A, Waisfisz Q, Wharton J, Wort SJ, Ouwehand WH, Soranzo N, Lawrie A, Upton PD, Wilkins MR, Trembath RC, Morrell NW. Identification of rare sequence variation underlying heritable pulmonary arterial hypertension. Nat Commun. 2018;9(1):1416.PMID: 29650961
***Kiskin FN, Chang CH, Huang CJZ, Kwieder B, Cheung C, Dunmore BJ, Sinha S, Morrell NW, Rana AA. Contributions of BMPR2 mutations and extrinsic factors to cellular phenotypes of pulmonary arterial hypertension revealed by iPSC modelling. Am J Respir Crit Care Med 2018;198(2):271-275. PMID: 29547009
***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, Newnham M, 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; NIHR BioResource–Rare Diseases Consortium; UK National Cohort Study of Idiopathic and Heritable PAH, 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. PMID: 28972005
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. PMID: 28972001
***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. PMID: 28971999
Dean A, Gegorc T, Docherty CK, Harvey KY, Nilsen M, Morrell NW, MacLean MR. Role of the Aryl Hydrocarbon Receptor in Sugen 5416-induced Experimental Hypertension. Am J Respir Cell Mol Biol 2018;58(3):320-330. PMID: 28956952
2017
***Mitrofan CG, Appleby SL, Nash GB, Mallat Z, Chilvers ER, Upton PD, Morrell NW. Bone morphogenetic protein 9 (BMP9) and BMP10 enhance tumor necrosis factor-α-induced monocyte recruitment to the vascular endothelium mainly via activing receptor-like kinase 2. J Biol Chem2017;292:13714-13726. PMID: 28646109
Yang, P, Read, C, Kuc RE, Buonincontri G, Southwood M, Torella R, Upton PD, Crosby A, Sawiak SJ, Carpenter TA, Glen RC, Morrell NW, Maguire JJ, Davenport AP. Elabela/Toddler is an Endogenous Agonist of the Apelin APJ receptor in the adult cardiovascular system, and exogenous administration of the peptide compensates for the downregulation of its expression in pulmonary arterial hypertension. Circulation 2017:135(12):1160-1173. PMID: 28137936
***Hurst LA, Dunmore BJ, Long L, Crosby A, Al-Lamki R, Deighton J, Southwood M, Yang X, Nikolic MZ, Herera B, Inman CJ, Bradley JR, Rana AA, Upton PD, Morrell NW. TNFα drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signaling. Nat Commun 2017;13;8:14079. PMID: 28084316
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 JS, Lawrie A, Graf S, Morrell NW, Wilkins MR. Plasma metabolomics implicates modified transfer RNAs and altered bioenergetics in the outcomes of pulmonary arterial hypertension. Circulation 2017;135(5):460-475. PMID: 27881557
2016
Appleby SL, Mitrofan CG, Crosby A, Hoenderdos K, Lodge K, Upton PD, Yates CM, Nash GB, Chilvers ER, Morrell NW. Bone morphogenetic protein 9 enhances Lipopolysaccharide-induced leukocyte recruitment to the vascular endothelium. J Immunol 2016;197(8):3302-3314. PMID: 27647829
Li W, Salmon RM, Jiang H, Morrell NW. Regulation of the ALK1 ligands, BMP9 and BMP10. Biochem Soc Trans 2016;44(4):1135-41. PMID: 27528761
Cowburn AS, Crosby A, Macias D, Branco C, Colaço RD, Southwood M, Toshner M, Crotty Alexander LE, Morrell NW, Chilvers ER, Johnson RS. HIF2α-arginase axis is essential for the development of pulmonary hypertension. Proc Natl Acad Sci USA 2016;113(31):8801-6. PMID: 27432976
Zhao J, Mitrofan CG, Appleby SL, Morrell NW, Lever AM. Disrupted endothelial cell layer and exposed extracellular matrix proteins promote capture of late outgrowth endothelial progenitor cells. Stem Cells Int 2016;2016:1406304. PMID: 27413378
Rothman AM, Arnold ND, Pickworth JA, Iremonger J, Ciuclan L, Allen RM, Guth-Gundel S, Southwood M, Morrell NW, Thomas M, Francis SE, Rowlands DJ, Lawrie A. MicroRNA-140-5p and SMURF1 regulate pulmonary arterial hypertension. J Clin Invest 2016;126(7):2495-508. PMID: 27214554
Stevens HC, Deng L, Grant JS, Pinel K, Thomas M, Morrell NW, MacLean MR, Baker AH, Denby L. Regulation and function of miR-214 in pulmonary arterial hypertension. Pulm Circ2016;6(1):109-17. PMID: 27162619
***Evans JD, Girerd B, Montani D, Wang XJ, Galie N, Austin ED, Elliott G, Asano K, Grunig E, Yan Y, Jing ZC, Manes A, Palazzini M, Wheeler LA, Nakayama I, Satoh T, Eichstaedt C, Hinderhofer K, Wolf M, Rosenzweig EB, Chung WK,Soubrier F, Simonneau G, Sitbon O, Graf S, Kaptoge S, Di Angelantonio E, Humber M, Morrell NW. BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis. Lancet Respir Med2016;4(2):129-37. PMID: 26795434
Jiang H, Salmon RM, Upton PD, Wei Z, Lawera A, Daenport AP, Morrell NW, Li W. The prodomain-bound form of bone morphogenetic protein 10 is biologically active on endothelial cells. J Biol Chem 2016;291(6):2954-66. PMID: 26631724
Morrell NW, Bloch DB, ten Dijke P, Goumans MJ, Hata A, Smith J, Yu PB, Bloch KD. Targeting BMP signalling in cardiovascular disease and anaemia. Nat Rev Cardiol. 2016;13(2):106-20.PMID: 26461965
2015
Machado RD, Southgate L, Eichstaedt CA, Aldred MA, Austin ED, Best DH, Chung WK, Benjamin N, Elliott CG, Eyries M, Fischer C, Gräf S, Hinderhofer K, Humbert M, Keiles SB, Loyd JE, Morrell NW, Newman JH, Soubrier F, Trembath RC, Viales RR, Grünig E. Pulmonary Arterial Hypertension: A current perspective on established and emerging molecular genetic defects. Hum Mutat. 2015;36:1113-27. PMID: 26387786
Deng L, Blanco FJ, Stevens H, Lu R, Caudrillier A, McBride M, McClure JD, Grant J, Thomas M, Frid M, Stenmark K, White K, Seto AG, Morrell NW, Bradshaw AC, MacLean MR, Baker AH. MicroRNA-143 activation regulates smooth muscle and endothelial cell crosstalk in pulmonary arterial hypertension. Circ Res 2015;117:870-83. PMID: 26311719
Crosby A, Soon E, Jones FM, Southwood MR, Haghighat L, Toshner MR, Raine T, Horan I, Yang P, Moore S, Ferrer E, Wright P, Ormiston ML, White RJ, Haight DA, Dunne DW, Morrell NW. Hepatic shunting of eggs and pulmonary vascular remodeling in BMPR2 (+/-) mice with schistosomiasis. Am J Respir Crit Care Med 2015;192:355-65. PMID: 26308618
Frost AE, Barst RJ, Hoeper MM, Chang HJ, Frantz RP, Fukumoto Y, Galié N, Hassoun PM, Klose H, Matsubara H, Morrell NW, Peacock AJ, Pfeifer M, Simonneau G, Tapson VF, Torres F, Dario Vizza C, Lawrence D, Yang W, Felser JM, Quinn DA, Ghofrani HA. Long-term safety and efficacy of Imatinib in pulmonary arterial hypertension. J Heart Lung Transplant 2015;34:1366-75. PMID: 26210752
***Long L, Ormiston ML, Yang X, Southwood M, Graf S, Machado RD, Mueller M, Kinzel B, Yung LM, Wilkinson JM, Moore JD, Drake KM, Aldred MA, Yu PB, Upton PB, Morrell NW. Selective enhancement of endothelial BMPR-II with BMP9 reserves pulmonary arterial hypertension. Nat Med 2015;21:777-85. PMID: 26076038
***Soon E, Crosby A, Southwood M, Yang P, Tajsic T, Toshner M, Appleby S, Shanahan CM, Bloch KD, Pepke-Zaba J, Upton P, Morrell NW. BMPR-II deficiency promotes pulmonary hypertension via increased inflammatory cytokine production. Am J Respir Crit Care Med2015;192(7):859-72. PMID: 26073741
Wallace E, Morrell NW, Yang XD, Long L, Stevens H, Nilsen M, Loughlin L, Mair KM, Baker AH, MacLean MR. A sex-specific MicroRNA-96/5-Hydroxytryptamine 1B Axis influences development of pulmonary hypertension. Am J Respir Crit Care Med 2015;191:1432-42. PMID: 25871906
Mair KM, Yang XD, Long L, White K, Wallace E, Ewart MA, Docherty CK, Morrell NW, MacLean MR. Sex Affects BMPR-II Signalling in Pulmonary Artery Smooth Muscle Cells. Am J Respir Crit Care Med. 2015;191(6):693-703. PMID: 25608111
Davies RJ, Holmes AM, Deighton J, Long L, Yang X, Barker L, Walker C, Budd DC, Upton PD, Morrell NW. BMP type II receptor deficiency confers resistance to growth inhibition by TGF-β in pulmonary artery smooth muscle cells: role of proinflammatory cytokines. Am J Physiol Lung Cell Mol Physiol. 2012;302(6):L604-15.
Ormiston ML, Chang C, Long LL, Soon E, Jones D, Machado R, Treacy C, Toshner MR, Campbell K, Riding A, Southwood M, Pepke-Zaba J, Exley A, Trembath RC, Colucci F, Wills M, Trowsdale J, Morrell NW. Impaired natural killer cell phenotype and function in idiopathic and heritable pulmonary arterial hypertension. Circulation. 2012;126(9):1099-109.