Penn Pulmonary Vascular Cell Center

Cell Resources for the study of Pulmonary Arterial Hypertension (PAH)

Articles Related to this Resource and PHBI


  1. Crystal Woods, Sujatha Venkataraman, Robert Stearman, Leah Villegas, Russell Bowler, Tim McKinsey, Kaori Ihida-Stansbury, Mark Geraci, Kurt Stenmark, Frederick Domann,  Eva Nozik-Grayck. Regulation Of Aberrant Expression Of EC-SOD In Human Idiopathic Pulmonary Arterial Hypertension By Epigenetic Mechanisms. AJRCCM 2014 A3383

  2. Crystal Woods, Sujatha Venkataraman, Robert Stearman, Leah Villegas, Russell Bowler, Tim McKinsey, Kaori Ihida-Stansbury, Mark Geraci, Kurt Stenmark, Frederick Domann,  Eva Nozik-Grayck. Aberrant expression of EC-SOD in IPAH: possible regulation by epigenetic mechanisms. Grover Conference Sept. 2013

  3. Vitamin E attenuates P. aeruginosa -mediated injury in pulmonary endothelial cells. N. Anjum, K. E. Iles, M. G. Traber, J. Creighton,  S. Matalon, J. Pittet. FASEB J., 26: 1101.5, 2012.

  4. M. Jian, Z. Lemley, and J. Creighton.  AMP-activated Kinase (AMPK) isoforms selectively contribute to lung vascular repair. Am. J. Respir. Crit. Care Med., 2012.

  5. Ming-Yuan Jian and Judy Creighton.  Endostatin limits pulmonary endothelial repair via RhoA inactivation. FASEB J April 9, 2013 27:724.10

  6. Ming-Yuan Jian and Judy Creighton.  N-cadherin contributes to the pulmonary microvascular endothelial response to LPS. FASEB J April 9, 2013 27:724.12

  7. Ming-Yuan Jian and Judy Creighton.  Endogenous endostatin limits endothelial repair in lung vascular injury. Aspen Lung Conference. June 6, 2014.

  8. Ming-Yuan Jiang, Tao Yu, Paul Wolkowicz, and Judy Creighton . N-cadherin and AMPKα 1 contribute to the pulmonary endothelial response to LPS. Aspen Lung Conference. June 8, 2014.

  9. Non-biased proteomics discovery of pulmonary artery hypertension biomarkers periostin and matrix metalloproteinase 9. Nies, MK, Yang, J, Fu, Z and Everett, AD . Johns Hopkins University, USA. PAH conference 2014, and CPVD conference. 2014.

  10. Epigenetic Profiling In Idiopathic Pulmonary Arterial Hypertension: Evidence Of Dysregulated Metabolism. Cheadle C, Boorgula M, Hassoun PM, Barnes KC, Gao L . Am J Respir Crit Care Med 189;2014:A3377.

  11. Genome-wide MicroRNA Expression Profiling Identifies Novel Targets in Lung Tissue from Patients with Pulmonary Arterial Hypertension.  Wu D, Talbot Jr C.C, Gao L . American Heart Association Scientific Sessions 2014, Chicago, IL. (Abstract submitted).


  1. Villegas L, Stidham T, Nozik-Grayck E . Antioxidant therapies for lung and pulmonary vascular diseases. Pulmonary & Respiratory Medicine . (under revision).

  2. Crystal Woods, Sujatha Venkataraman, Robert Stearman, Tim McKinsey, Gary Mouradian, Leah Villegas, Russell Bowler, Kaori Ihida-Stansbury, Mark Geraci, Kurt Stenmark, Frederick Domann, Eva Nozik-Grayck . Epigenetic regulation Of Aberrant Expression Of EC-SOD In Human Idiopathic Pulmonary Arterial Hypertension. (In preparation)

  3. Creighton J.  Transmitting mechanical signals. Focus on "Mechanical induction of GroupV phospholipase A2 causes lung inflammation and acute lung injury" Am J Physiol Lung Cell Mol Physiol.  Aug; 15; 305(4):L279-81. E pub Jun 28, 2013. PMID:23812631.

  4. Jian M-Y, Alexeyev MF, Wolkowicz PE, Zmijewski JW and Creighton J . “Metformin stimulated AMPKα 1 promotes microvascular repair in acute lung injury” Am J Physiol Lung Cell Mol Physiol.  Dec; 305(11):L844-55. Epub Oct 4, 2013. PMID:24097562.

  5. Erythropoietin upregulation in pulmonary arterial hypertension Vanesa A. Karamanian, Michael Harhay, Gregory R. Grant, Harold I. Palevsky, William E. Grizzle , Roham T. Zamanian, Kaori Ihida-Stansbury, Darren B. Taichman, Steven M. Kawut , and Peter L. Jones. Pulmonary Circulation , Vol. 4, No. 2 (June 2014), pp. 269-279. Article DOI:  10.1086/675990.Article. Stable URL : . PMID:  Not available

  6. Reduced BMPR2 expression induces GM-CSF translation and macrophage recruitment in humans and mice to exacerbate pulmonary hypertension. Sawada, H., T. Saito, N.P. Nickel, T.P. Alastalo, J.P. Glotzbach, R. Chan, L. Haghighat, G. Fuchs, M. Januszyk, A. Cao, Y.J. Lai, J. Perez Vde, Y.M. Kim, L. Wang, P.I. Chen, E. Spiekerkoetter, Y. Mitani, G.C. Gurtner, P. Sarnow, and M. Rabinovitch,  Journal of Experimental Medicine. 2014. 211(2): p. 263-80. ( PMCID: PMC3920564) [Available on 2014/8/10].

  7. High throughput screen identifies FK506 as an activator of BMPR2 signaling that reverses endothelial dysfunction and pulmonary hypertension Edda Spiekerkoetter E, Hopper R, Li CG, Sawada H, Haghighat R, Chan R, Haghighat L, de Jesus Perez V, Wang L, Reddy S, Solow-Cordero D, El-Bizri N, ten Dijke P, Beachy PA, Rabinovitch M.  Journal of Clinical Investigation. 2013 Aug 1;123(8):3600-13. (PMCID: PMC3726153).

  8. Blocking macrophage leukotriene b4 prevents endothelial injury and reverses pulmonary hypertension. Tian W, Jiang X, Tamosiuniene R, Sung YK, Qian J, Dhillon G, Gera L, Farkas L, Rabinovitch M, Zamanian RT, Inayathullah M, Fridlib M, Rajadas J, Peters-Golden M, Voelkel NF, Nicolls MR . Science Translational Medicine. 2013 Aug 28;5(200):200ra117. (PMCID: PMC4016764).

  9. Loss of adenomatous poliposis Coli-α 3 integrin interaction promotes endothelial apoptosis in mice and humans. de Jesus Perez VA, Yuan K, Orcholski ME, Sawada H, Zhao M, Li CG, Tojais NF, Nickel NP, Rajagopalan V, Spiekerkoetter E, Wang L, Dutta R, Bernstein D, Rabinovitch M.  Circulation Research. 2012 Dec 7;111(12):1551-64. (PMCID: PMC3821702). - sec-25 

  10. BMPR2 Protects Against Pulmonary Hypertension by Regulating P53, PGC1alpha and Tfam to Promote Mitochondrial DNA Integrity &Endothelian Survival. Diebold, I., H. J.K., G.C. Li, N.P. Nickel, M. Kaschwich, L. Wang, S. Reddy, K. Nakahira, M.A.A. Alcazar, and M. Rabinovitch (submitted manuscript, under revision).

  11. RNAseq Reveals a Novel Pathway of Endothelial Dysfunction in Pulmonary Arterial Hypertension. Rhodes CJ, Im H, Nickel NP, Cao A, Hopper R, Tojais NF, Wang L, Li CG, Chen P-I, Spiekerkoetter E, Xian Z, Chen R, Zhao MM, Kaschwich M, Wu J, Snyder MP, Rabinovitch M. (Manuscript in Preparation).  

  12. Elafin Reverses Experimental Pulmonary Hypertension Related to Elastase Inhibition & Caveolin-1- Dependent BMPR2 Signaling. Nickel NP, Spiekerkoetter E, Li CG, Li H, Kaschwich M, Diebold I, Hennings JK, Wang L, Rabinovitch M. (Manuscript in Preparation).

  13. Transglutaminase-2 mediates calcium-regulated crosslinking of the Y-Box 1 (YB-1) translation-regulatory protein in TGFβ 1-activated myofibroblasts. William L. Willis, Seethalakshmi Hariharan, Jason J. David and Arthur Roger Strauch* . Article first published online: J Cell Biochem. 2013 Dec;114(12):2753-69. doi: 10.1002/jcb.24624. PMID: 23804301.;jsessionid=13A867542B07DBAC2E9AB824601CAE6B.f02t02