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Chronic allergic inflammation causes vascular remodeling and pulmonary hypertension in BMPR2 hypomorph and wild-type mice.

Mushaben EM, Hershey GK, Pauciulo MW, Nichols WC, Le Cras TD.

PLoS One. 2012;7(3):e32468. doi: 10.1371/journal.pone.0032468. Epub 2012 Mar 9.


Modification of hemodynamic and immune responses to exposure with a weak antigen by the expression of a hypomorphic BMPR2 gene.

Park SH, Chen WC, Hoffman C, Marsh LM, West J, Grunig G.

PLoS One. 2013;8(1):e55180. doi: 10.1371/journal.pone.0055180. Epub 2013 Jan 29.


Bone morphogenetic protein receptor type II deficiency and increased inflammatory cytokine production. A gateway to pulmonary arterial hypertension.

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.

Am J Respir Crit Care Med. 2015 Oct 1;192(7):859-72. doi: 10.1164/rccm.201408-1509OC.


Serotonin increases susceptibility to pulmonary hypertension in BMPR2-deficient mice.

Long L, MacLean MR, Jeffery TK, Morecroft I, Yang X, Rudarakanchana N, Southwood M, James V, Trembath RC, Morrell NW.

Circ Res. 2006 Mar 31;98(6):818-27. Epub 2006 Feb 23.


Hepatic Shunting of Eggs and Pulmonary Vascular Remodeling in Bmpr2(+/-) Mice with Schistosomiasis.

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.

Am J Respir Crit Care Med. 2015 Dec 1;192(11):1355-65. doi: 10.1164/rccm.201412-2262OC.


Increased susceptibility to pulmonary hypertension in heterozygous BMPR2-mutant mice.

Song Y, Jones JE, Beppu H, Keaney JF Jr, Loscalzo J, Zhang YY.

Circulation. 2005 Jul 26;112(4):553-62. Epub 2005 Jul 18.


Neutralization of TSLP inhibits airway remodeling in a murine model of allergic asthma induced by chronic exposure to house dust mite.

Chen ZG, Zhang TT, Li HT, Chen FH, Zou XL, Ji JZ, Chen H.

PLoS One. 2013;8(1):e51268. doi: 10.1371/journal.pone.0051268. Epub 2013 Jan 2.


Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice.

Song Y, Coleman L, Shi J, Beppu H, Sato K, Walsh K, Loscalzo J, Zhang YY.

Am J Physiol Heart Circ Physiol. 2008 Aug;295(2):H677-90. doi: 10.1152/ajpheart.91519.2007. Epub 2008 Jun 13. Erratum in: Am J Physiol Heart Circ Physiol. 2009 Oct;297(4):H1544.


Physiologic and molecular consequences of endothelial Bmpr2 mutation.

Majka S, Hagen M, Blackwell T, Harral J, Johnson JA, Gendron R, Paradis H, Crona D, Loyd JE, Nozik-Grayck E, Stenmark KR, West J.

Respir Res. 2011 Jun 22;12:84. doi: 10.1186/1465-9921-12-84.


Endothelin-Bone morphogenetic protein type 2 receptor interaction induces pulmonary artery smooth muscle cell hyperplasia in pulmonary arterial hypertension.

Maruyama H, Dewachter C, Belhaj A, Rondelet B, Sakai S, Remmelink M, Vachiery JL, Naeije R, Dewachter L.

J Heart Lung Transplant. 2015 Mar;34(3):468-78. doi: 10.1016/j.healun.2014.09.011. Epub 2014 Sep 28.


SMAD1 deficiency in either endothelial or smooth muscle cells can predispose mice to pulmonary hypertension.

Han C, Hong KH, Kim YH, Kim MJ, Song C, Kim MJ, Kim SJ, Raizada MK, Oh SP.

Hypertension. 2013 May;61(5):1044-52. doi: 10.1161/HYPERTENSIONAHA.111.199158. Epub 2013 Mar 11.


Raf/ERK drives the proliferative and invasive phenotype of BMPR2-silenced pulmonary artery endothelial cells.

Awad KS, Elinoff JM, Wang S, Gairhe S, Ferreyra GA, Cai R, Sun J, Solomon MA, Danner RL.

Am J Physiol Lung Cell Mol Physiol. 2016 Jan 15;310(2):L187-201. doi: 10.1152/ajplung.00303.2015. Epub 2015 Nov 20.


Penetrance of pulmonary arterial hypertension is modulated by the expression of normal BMPR2 allele.

Hamid R, Cogan JD, Hedges LK, Austin E, Phillips JA 3rd, Newman JH, Loyd JE.

Hum Mutat. 2009 Apr;30(4):649-54. doi: 10.1002/humu.20922.


Rescuing the BMPR2 signaling axis in pulmonary arterial hypertension.

West J, Austin E, Fessel JP, Loyd J, Hamid R.

Drug Discov Today. 2014 Aug;19(8):1241-5. doi: 10.1016/j.drudis.2014.04.015. Epub 2014 May 2. Review.


Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension.

Hong KH, Lee YJ, Lee E, Park SO, Han C, Beppu H, Li E, Raizada MK, Bloch KD, Oh SP.

Circulation. 2008 Aug 12;118(7):722-30. doi: 10.1161/CIRCULATIONAHA.107.736801. Epub 2008 Jul 28.


Concurrent dual allergen exposure and its effects on airway hyperresponsiveness, inflammation and remodeling in mice.

DiGiovanni FA, Ellis R, Wattie J, Hirota JA, Southam DS, Inman MD.

Dis Model Mech. 2009 May-Jun;2(5-6):275-82. doi: 10.1242/dmm.001859. Epub 2009 Apr 6.


BMPR2 gene delivery reduces mutation-related PAH and counteracts TGF-β-mediated pulmonary cell signalling.

Feng F, Harper RL, Reynolds PN.

Respirology. 2016 Apr;21(3):526-32. doi: 10.1111/resp.12712. Epub 2015 Dec 21.


Transforming growth factor-beta regulates house dust mite-induced allergic airway inflammation but not airway remodeling.

Fattouh R, Midence NG, Arias K, Johnson JR, Walker TD, Goncharova S, Souza KP, Gregory RC Jr, Lonning S, Gauldie J, Jordana M.

Am J Respir Crit Care Med. 2008 Mar 15;177(6):593-603. doi: 10.1164/rccm.200706-958OC. Epub 2008 Jan 3.


Serotonin 2B Receptor Antagonism Prevents Heritable Pulmonary Arterial Hypertension.

West JD, Carrier EJ, Bloodworth NC, Schroer AK, Chen P, Ryzhova LM, Gladson S, Shay S, Hutcheson JD, Merryman WD.

PLoS One. 2016 Feb 10;11(2):e0148657. doi: 10.1371/journal.pone.0148657. eCollection 2016.


Investigation of second genetic hits at the BMPR2 locus as a modulator of disease progression in familial pulmonary arterial hypertension.

Machado RD, James V, Southwood M, Harrison RE, Atkinson C, Stewart S, Morrell NW, Trembath RC, Aldred MA.

Circulation. 2005 Feb 8;111(5):607-13.

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