Therapeutics Targeting of Dysregulated Redox Equilibrium and Endothelial Dysfunction

Stimulation of tetrahydrobiopterin synthesis induced by insulin: possible involvement of phosphatidylinositol 3-kinase

Ishii, M., Shimizu, S., Nagai, T., Shiota, K., Kiuchi, Y., & Yamamoto, T. (2001) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/s1357-2725(00)00070-4 · Cited by 54

Tetrahydrobiopterin: pleiotropic roles in cardiovascular pathophysiology

Cunnington, C., & Channon, K. M. (2010) · Heart · doi:10.1136/hrt.2009.180430 · Cited by 52

Role of Mitochondrial Aldehyde Dehydrogenase in Nitroglycerin-Induced Vasodilation of Coronary and Systemic Vessels

Zhang, J., Chen, Z., Cobb, F. R., & Stamler, J. S. (2004) · Circulation · doi:10.1161/01.cir.0000138105.17864.6b · Cited by 52

Oxidative stress in patients with COPD and pulmonary hypertension

Joppa, P., Petrášová, D., Stancak, B., Dorková, Z., & Tkáčová, R. (2007) · Wiener klinische Wochenschrift · doi:10.1007/s00508-007-0819-y · Cited by 50

Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice

Dasgupta, T., Fabry, M. E., & Kaul, D. K. (2009) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.00611.2009 · Cited by 50

Suppression of eNOS-derived superoxide by caveolin-1: a biopterin-dependent mechanism

Karuppiah, K. M., Druhan, L. J., Chen, C., Smith, T., Zweier, J. L., Sessa, W. C., & Cardounel, A. J. (2011) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.00936.2010 · Cited by 49

Decreased Whole Body Endogenous Nitric Oxide Production in Patients with Primary Pulmonary Hypertension

Demoncheaux, E. A. G., Higenbottam, T., Kiely, D. G., Wong, J.-M., Wharton, S., Varcoe, R. W., Siddons, T., Spivey, A. C., Hall, K., & Giże, A. P. (2005) · Journal of Vascular Research · doi:10.1159/000083502 · Cited by 48

Effect of PPARγ inhibition on pulmonary endothelial cell gene expression: gene profiling in pulmonary hypertension

Tian, J., Smith, A., Nechtman, J., Podolsky, R. H., Aggarwal, S., Snead, C. M., Kumar, S., Elgaish, M., Oishi, P. E., Goerlach, A., et al. (2009) · Physiological Genomics · doi:10.1152/physiolgenomics.00094.2009 · Cited by 48

Safety of sapropterin dihydrochloride (6r–bh4) in patients with pulmonary hypertension

Robbins, I. M., Hemnes, A. R., Simon Gibbs, J., Christman, B. W., Howard, L., Meehan, S., Cabrita, I., Gonzalez, R., Oyler, T., Zhao, L., et al. (2011) · Experimental Lung Research · doi:10.3109/01902148.2010.512972 · Cited by 47

PPARγ as a potential therapeutic target in pulmonary hypertension

Sutliff, R. L., Kang, B., & Hart, C. M. (2010) · Therapeutic Advances in Respiratory Disease · doi:10.1177/1753465809369619 · Cited by 44

l -Arginine promotes angiogenesis in the chronically hypoxic lung: a novel mechanism ameliorating pulmonary hypertension

Howell, K., Costello, C. M., Sands, M. A., Dooley, I. J., & McLoughlin, P. (2009) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.90327.2008 · Cited by 44

Inhibition of p38 MAPK reverses hypoxia-induced pulmonary artery endothelial dysfunction

Weerackody, R. P., Welsh, D., Wadsworth, R. M., & Peacock, A. J. (2009) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.00977.2008 · Cited by 44

Increased xanthine oxidase activity in idiopathic pulmonary arterial hypertension

Spiekermann, S., Schenk, K., & Hoeper, M. M. (2009) · European Respiratory Journal · doi:10.1183/09031936.00013309 · Cited by 43

Effects of Nitroglycerin on Soluble Guanylate Cyclase

Artz, J. D., Schmidt, B., McCracken, J., & Marletta, M. A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.c200170200 · Cited by 40

The NOX on Pulmonary Hypertension

Sanders, K. A., & Hoidal, J. R. (2007) · Circulation Research · doi:10.1161/circresaha.107.158246 · Cited by 39

Sepiapterin improves angiogenesis of pulmonary artery endothelial cells with in utero pulmonary hypertension by recoupling endothelial nitric oxide synthase

Teng, R., Du, J., Xu, H., Bakhutashvili, I., Eis, A., Shi, Y., Pritchard, K. A., & Konduri, G. G. (2011) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00316.2010 · Cited by 38

Modulation of Inducible Nitric Oxide Synthase by Hypoxia in Pulmonary Artery Endothelial Cells

Zulueta, J. J., Sawhney, R., Kayyali, U. S., Fogel, M. A., Donaldson, C., Huang, H., Lanzillo, J. J., & Hassoun, P. M. (2002) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/ajrcmb.26.1.4510 · Cited by 36

Peroxynitrite stimulates pulmonary artery endothelial and smooth muscle cell proliferation: Involvement of ERK and PKC

Agbani, E. O., Coats, P., Mills, A. A., & Wadsworth, R. M. (2010) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2010.09.003 · Cited by 34

The Emerging Role of Nitrite as an Endogenous Modulator and Therapeutic Agent of Cardiovascular Function

Tota, B., Quintieri, A. M., & Angelone, T. (2010) · Current Medicinal Chemistry · doi:10.2174/092986710791163948 · Cited by 33

Plasma concentrations of asymmetric dimethylarginine, an endogenous nitric oxide synthase inhibitor, are elevated in sickle cell patients but do not increase further during painful crisis

Landburg, P. P., Teerlink, T., Muskiet, F. A. J., Duits, A. J., & Schnog, J. B. (2008) · American Journal of Hematology · doi:10.1002/ajh.21184 · Cited by 30

Adenosine deaminase–adenosine pathway in hemolysis-associated pulmonary hypertension

Tofovic, S. P., Jackson, E. K., & Rafikova, O. (2009) · Medical Hypotheses · doi:10.1016/j.mehy.2008.12.043 · Cited by 27

Rosuvastatin ameliorates the development of pulmonary arterial hypertension in the transgenic (mRen2)27 rat

DeMarco, V. G., Habibi, J., Whaley‐Connell, A. T., Schneider, R. I., Sowers, J. R., Andresen, B. T., Gutweiler, A. A., Ma, L., Johnson, M. S., Ferrario, C. M., et al. (2009) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.00048.2009 · Cited by 27

Echocardiographic findings in patients with sickle cell disease

Naoman, S. G., Nouraie, M. S., Castro, O. L., Nwokolo, C., Fadojutimi-Akinsiku, M., Diaz, S. F., Willie-Carnegie, G., Andrews, N., & Gordeuk, V. R. (2009) · Annals of Hematology · doi:10.1007/s00277-009-0764-0 · Cited by 25

Umbilical Arterial S-nitrosothiols in Stressed Newborns: Role in Perinatal Circulatory Transition

Gaston, B. M., Fry, E., Sears, S., Heroman, W. M., Ignarro, L. J., & Stamler, J. S. (1998) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1998.9865 · Cited by 24

Tetrahydrobiopterin and the regulation of hypoxic pulmonary vasoconstriction

Francis, B. N., Wilkins, M. R., & Zhao, L. (2010) · European Respiratory Journal · doi:10.1183/09031936.00188809 · Cited by 23

Alterations in lung arginine metabolism in lambs with pulmonary hypertension associated with increased pulmonary blood flow

Sharma, S., Kumar, S., Sud, N., Wiseman, D. A., Tian, J., Rehmani, I., Datar, S. A., Oishi, P. E., Fratz, S., Venema, R. C., et al. (2009) · Vascular Pharmacology · doi:10.1016/j.vph.2009.09.005 · Cited by 19

Corrigendum to “Endothelial nitric oxide (NO) and its pathophysiologic regulation” [Vascular Pharmacology 49 (2008) 134–140]

Chatterjee, A., Black, S. M., & Catravas, J. D. (2009) · Vascular Pharmacology · doi:10.1016/j.vph.2009.02.001 · Cited by 19

Increased Nitrosoglutathione Reductase Activity in Hypoxic Pulmonary Hypertension in Mice

Wu, X., Du, L., Xu, X., Tan, L., & Li, R. (2010) · Journal of Pharmacological Sciences · doi:10.1254/jphs.09279fp · Cited by 19

Response to Inhaled Nitric Oxide Predicts Survival in Patients With Pulmonary Hypertension

Krasuski, R. A., Devendra, G. P., Hart, S. A., Wang, A., Harrison, J. K., & Bashore, T. M. (2011) · Journal of Cardiac Failure · doi:10.1016/j.cardfail.2010.11.010 · Cited by 19

Massive quantities of asymmetric dimethylarginine (ADMA) are incorporated in red blood cell proteins and may be released by proteolysis following hemolytic stress

D’Alecy, L. G., & Billecke, S. S. (2010) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2010.03.004 · Cited by 16

Tetrahydrobiopterin analogues with NO-dependent pulmonary vasodilator properties

Kunuthur, S. P., Milliken, P. H., Gibson, C. L., Suckling, C. J., & Wadsworth, R. M. (2010) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2010.09.070 · Cited by 15

L-arginine plasma levels and severity of idiopathic pulmonary arterial hypertension

Beyer, Kolditz, M., Ewert, Rubens, Opitz, Schellong, Hoeffken, & Halank (2008) · VASA · doi:10.1024/0301-1526.37.1.61 · Cited by 14

Inhaled nitric oxide in adults with the acute respiratory distress syndrome

Markewitz, B. A., & Michael, J. R. (2000) · Respiratory Medicine · doi:10.1053/rmed.2000.0928 · Cited by 6

Pathophysiology of ischaemia-reperfusion injury

Carden, D. L., & Granger, D. N. (2000) · The Journal of Pathology · doi:10.1002/(sici)1096-9896(200002)190:3 · Cited by 5

NT-Pro Brain Natriuretic Peptide Levels and the Risk of Stroke and Death in the Cooperative Study of Sickle Cell Disease.

Machado, R. F., Hildesheim, M. E., Mendelsohn, L. G., Kato, G. J., & Gladwin, M. T. (2009) · Blood · doi:10.1182/blood.v114.22.1541.1541 · Cited by 5

Sources of Vascular Nitric Oxide and Reactive Oxygen Species and Their Regulation

Tejero, J., Shiva, S., & Gladwin, M. T. (2019) · Physiological Reviews · doi:10.1152/physrev.00036.2017 · Cited by 541

Targeting vascular (endothelial) dysfunction

Daiber, A., Steven, S., Weber, A., Shuvaev, V. V., Muzykantov, V. R., Laher, I., Li, H., Lamas, S., & Münzel, T. (2017) · British Journal of Pharmacology · doi:10.1111/bph.13517 · Cited by 501

Inflammation in sickle cell disease

Conran, N., & Belcher, J. D. (2018) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-189012 · Cited by 316

Crosstalk of mitochondria with NADPH oxidase via reactive oxygen and nitrogen species signalling and its role for vascular function

Daiber, A., Lisa, F. D., Oelze, M. W., Kröller‐Schön, S., Steven, S., Schulz, E., & Münzel, T. F. (2015) · British Journal of Pharmacology · doi:10.1111/bph.13403 · Cited by 274

Regulation of obesity and insulin resistance by nitric oxide

Sansbury, B. E., & Hill, B. G. (2014) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2014.05.016 · Cited by 274

Endothelial Redox Dysregulation in Pulmonary Arterial Hypertension: Compartmentalized Mechanisms and Therapeutic Implications

Moreno-Caceres, S., & Marciano, D. P. (2026) · International Journal of Molecular Sciences · doi:10.3390/ijms27209096

Reactive oxygen and nitrogen species in pulmonary hypertension

Tabima, D. M., Frizzell, S. A., & Gladwin, M. T. (2012) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2012.02.041 · Cited by 194

Redox Regulation of Lung Endothelial Perk, Unfolded Protein Response (Upr) and Proliferation Via Nox1: Targeted Inhibition as a Potential Therapy for PahPreprint

Goossen, C., Kufner, A., Dustin, C. M., Ghouleh, I. A., Yuan, S. S., Straub, A. C., Sembrat, J., Baust, J., Gomez, D. A., Kračun, D., et al. (2025) · SSRN Electronic Journal · doi:10.2139/ssrn.5064708

Oxidative and nitrosative signalling in pulmonary arterial hypertension — Implications for development of novel therapies

Hansen, T., Galougahi, K. K., Celermajer, D. S., Rasko, N. B., Tang, O., Bubb, K. J., & Figtree, G. A. (2016) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2016.05.005 · Cited by 48

Concealed role of red blood cells in pathogenesis of pulmonary arterial hypertension: Decreased red blood cell nitric oxide generation and effect of Rho-Kinase inhibitor fasudil

Başarıcı, İ., Özen, N., Kilavuz, E., Kısak, F., Basralı, F., Yaraş, N., Köksoy, S., Çelik, M. L., & Ülker, P. (2020) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-200892 · Cited by 10