Bhattacharyya S, Wei J, Varga J. Understanding fibrosis in systemic sclerosis: shifting paradigms, emerging opportunities. Nat Rev Rheumatol. 2011;8:42–54.
Article
PubMed Central
PubMed
Google Scholar
Kim KK, Kugler MC, Wolters PJ, Robillard L, Galvez MG, Brumwell AN, et al. Alveolar epithelial cell mesenchymal transition develops in vivo during pulmonary fibrosis and is regulated by the extracellular matrix. Proc Natl Acad Sci U S A. 2006;103:13180–5.
Article
PubMed Central
CAS
PubMed
Google Scholar
Wu Z, Yang L, Cai L, Zhang M, Cheng X, Yang X, et al. Detection of epithelial to mesenchymal transition in airways of a bleomycin induced pulmonary fibrosis model derived from an alpha-smooth muscle actin-Cre transgenic mouse. Respir Res. 2007;8:1.
Article
PubMed Central
CAS
PubMed
Google Scholar
Hashimoto N, Phan SH, Imaizumi K, Matsuo M, Nakashima H, Kawabe T, et al. Endothelial mesenchymal transition in bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol. 2010;43:161–72.
Article
PubMed Central
CAS
PubMed
Google Scholar
Cipriani P, Marrelli A, Di Benedetto P, Liakouli V, Carubbi F, Ruscitti P, et al. Scleroderma mesenchymal stem cells display a different phenotype from healthy controls; implications for regenerative medicine. Angiogenesis. 2013;16:595–607.
Article
CAS
PubMed
Google Scholar
Cipriani P, Di Benedetto P, Ruscitti P, Campese AF, Liakouli V, Carubbi F, et al. Impaired endothelium-mesenchymal stem cells cross-talk in systemic sclerosis: a link between vascular and fibrotic features. Arthritis Res Ther. 2014;16:442.
Article
PubMed Central
PubMed
Google Scholar
Cipriani P, Di Benedetto P, Capece D, Zazzeroni F, Liakouli V, Ruscitti P, et al. Impaired Cav-1 expression in SSc mesenchymal cells upregulates VEGF signaling: a link between vascular involvement and fibrosis. Fibrogenesis Tissue Repair. 2014;7:13.
Article
PubMed Central
PubMed
Google Scholar
Barton M, Yanagisawa M. Endothelin: 20 years from discovery to therapy. Can J Physiol Pharmacol. 2008;86:485–98.
Article
CAS
PubMed
Google Scholar
Inoue A, Yanagisawa M, Kimura S, Kasuya Y, Miyauchi T, Goto K, et al. The human endothelin family: three structurally and pharmacologically distinct isopeptides predicted by three separate genes. Proc Natl Acad Sci U S A. 1989;86:2863–67.
Article
PubMed Central
CAS
PubMed
Google Scholar
Watts S. Endothelin receptors: what’s new and what do we need to know? Am J Physiol Regul Integr Comp Physiol. 2010;298:R254–60.
Article
PubMed Central
CAS
PubMed
Google Scholar
Raja SG. Endothelin receptor antagonists for pulmonary arterial hypertension: an overview. Cardiovasc Ther. 2010;28:e65–71.
Article
CAS
PubMed
Google Scholar
Razzaq Z, Hussain M, Naqvi S, Aslam M. Correlation of plasma endothelin-1 levels with pulmonary hypertension after inhaled nitric oxide therapy. J Ayub Med Coll Abbottabad. 2009;21:106–10.
PubMed
Google Scholar
Teder P, Noble PW. A cytokine reborn? Endothelin-1 in pulmonary inflammation and fibrosis. Am J Respir Cell Mol Biol. 2000;23:7–10.
Article
CAS
PubMed
Google Scholar
Horstmeyer A, Licht C, Scherr G, Eckes B, Krieg T. Signalling and regulation of collagen I synthesis by ET-1 and TGF-beta1. FEBS J. 2005;272:6297–309.
Article
CAS
PubMed
Google Scholar
Lagares D, García-Fernández RA, Jiménez CL, Magán-Marchal N, Busnadiego O, Lamas S, et al. Endothelin 1 contributes to the effect of transforming growth factor beta1 on wound repair and skin fibrosis. Arthritis Rheum. 2010;62:878–89.
Article
CAS
PubMed
Google Scholar
Goumans MJ, Valdimarsdottir G, Itoh S, Lebrin F, Larsson J, Mummery C, et al. Activin receptor-like kinase (ALK)1 is an antagonistic mediator of lateral TGFβ/ALK5 signaling. Mol Cell. 2003;12:817–28.
Article
CAS
PubMed
Google Scholar
Goumans MJ, Valdimarsdottir G, Itoh S, Rosendahl A, Sideras P, ten Dijke P. Balancing the activation state of the endothelium via two distinct TGFβ type I receptors. EMBO J. 2002;21:1743–53.
Article
PubMed Central
CAS
PubMed
Google Scholar
Daly AC, Randall RA, Hill CS. Transforming growth factor beta-induced Smad1/5 phosphorylation in epithelial cells is mediated by novel receptor complexes and is essential for anchorage-independent growth. Mol Cell Biol. 2008;28:6889–902.
Article
PubMed Central
CAS
PubMed
Google Scholar
Leask A. Targeting the TGFbeta, endothelin-1 and CCN2 axis to combat fibrosis in scleroderma. Cell Signal. 2008;20:1409–14.
Article
CAS
PubMed
Google Scholar
Cutolo M, Montagna P, Brizzolara R, Smith V, Alessandri E, Villaggio B, et al. Effects of macitentan and its active metabolite on cultured human systemic sclerosis and control skin fibroblasts. J Rheumatol. 2015;42:456–63.
Iglarz M, Bossu A, Wanner D, Bortolamiol C, Rey M, Hess P, et al. Comparison of pharmacological activity of macitentan and bosentan in preclinical models of systemic and pulmonary hypertension. Life Sci. 2014;118:333–9.
Article
CAS
PubMed
Google Scholar
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65:2737–47.
Article
PubMed Central
PubMed
Google Scholar
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72:1747–55.
Article
PubMed
Google Scholar
Bhattacharyya S, Wei J, Tourtellotte WG, Hinchcliff M, Gottardi CG, Varga J. Fibrosis in systemic sclerosis: common and unique pathobiology. Fibrogenesis Tissue Repair. 2012;6:5. S18.
Google Scholar
Chen Y, Shi-wen X, Eastwood M, Black CM, Denton CP, Leask A, et al. Contribution of activin receptor-like kinase 5 (transforming growth factor beta receptor type I) signalling to the fibrotic phenotype of scleroderma fibroblasts. Arthritis Rheum. 2006;54:1309–16.
Article
CAS
PubMed
Google Scholar
Leask A, Abraham DJ. TGFβ signaling and the fibrotic response. FASEB J. 2004;18:816–27.
Article
CAS
PubMed
Google Scholar
Denton CP, Abraham DJ. Transforming growth factor-β and connective tissue growth factor: key cytokines in scleroderma pathogenesis. Curr Opin Rheumatol. 2001;13:505–11.
Article
CAS
PubMed
Google Scholar
Border WA, Noble NA. Transforming growth factor β in tissue fibrosis. N Engl J Med. 1994;331:1286–92.
Article
CAS
PubMed
Google Scholar
Akamata K, Asano Y, Aozasa N, Noda S, Taniguchi T, Takahashi T, et al. Bosentan reverses the profibrotic phenotype of systemic sclerosis dermal fibroblasts via increasing DNA binding ability of transcription factor Fli1. Arthritis Res Ther. 2014;16:R86.
Article
PubMed Central
PubMed
Google Scholar
Little PJ, Burch ML, Al-Aryahi S, Zheng W. The paradigm of G protein receptor transactivation: a mechanistic definition and novel example. Sci World J. 2011;11:709–14.
Article
CAS
Google Scholar
Burch ML, Ballinger ML, Yang SN, Getachew R, Itman C, Loveland K, et al. Thrombin stimulation of proteoglycan synthesis in vascular smooth muscle is mediated by protease-activated receptor-1 transactivation of the transforming growth factor β type I receptor. J Biol Chem. 2010;285:26798–805.
Article
PubMed Central
CAS
PubMed
Google Scholar
Shi-Wen X, Chen Y, Denton CP, Eastwood M, Renzoni EA, Bou-Gharios G, et al. Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Biol Cell. 2004;15:2707–19.
Article
PubMed Central
PubMed
Google Scholar
Shi-Wen X, Rodríguez-Pascual F, Lamas S, Holmes A, Howat S, Pearson JD, et al. Constitutive ALK5-independent c-Jun N-terminal kinase activation contributes to endothelin-1 overexpression in pulmonary fibrosis: evidence of an autocrine endothelin loop operating through the endothelin A and B receptors. Mol Cell Biol. 2006;26:5518–27.
Article
PubMed Central
PubMed
Google Scholar
Shi-Wen X, Renzoni EA, Kennedy L, Howat S, Chen Y, Pearson JD, et al. Endogenous endothelin-1 signaling contributes to type I collagen and CCN2 overexpression in fibrotic fibroblasts. Matrix Biol. 2007;26:625–32.
Article
PubMed
Google Scholar
Shi-wen X, Kennedy L, Renzoni EA, Bou-Gharios G, du Bois RM, Black CM, et al. Endothelin is a downstream mediator of profibrotic responses to transforming growth factor b in human lung fibroblasts. Arthritis Rheum. 2007;56:4189–94.
Article
PubMed
Google Scholar
Natarajan K, Berk BC. Crosstalk coregulation mechanisms of G protein-coupled receptors and receptor tyrosine kinases. Methods Mol Biol. 2006;332:51–77.
CAS
PubMed
Google Scholar
Daub H, Weiss FU, Wallasch C, Ullrich A. Role of transactivation of the EGF receptor in signalling by G-protein-coupled receptors. Nature. 1996;379:557–60.
Article
CAS
PubMed
Google Scholar
Habib AA, Högnason T, Ren J, Stefánsson K, Ratan RR. The epidermal growth factor receptor associates with and recruits phosphatidylinositol 3-kinase to the platelet-derived growth factor beta receptor. J Biol Chem. 1998;273:6885–91.
Article
CAS
PubMed
Google Scholar
Alderton F, Rakhit S, Kong KC, Palmer T, Sambi B, Pyne S, et al. Tethering of the platelet-derived growth factor beta receptor to G-protein-coupled receptors. A novel platform for integrative signalling by these receptor classes in mammalian cells. J Biol Chem. 2001;276:28578–85.
Article
CAS
PubMed
Google Scholar
Karoor V, Wang L, Wang HY, Malbon CC. Insulin stimulates sequestration of beta-adrenergic receptors and enhanced association of beta-adrenergic receptors with Grb2 via tyrosine 350. J Biol Chem. 1998;273:33035–41.
Article
CAS
PubMed
Google Scholar
Wang H, Doronin S, Malbon CC. Insulin activation of mitogen-activated protein kinases Erk1,2 is amplified via beta-adrenergic receptor expression and requires the integrity of the Tyr350 of the receptor. J Biol Chem. 2000;275:36086–93.
Article
CAS
PubMed
Google Scholar
Badea I, Taylor M, Rosenberg A, Foldvari M. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis. Rheumatology (Oxford). 2009;48:213–21.
Article
CAS
Google Scholar
King Jr TE, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;177:75–81.
Article
CAS
PubMed
Google Scholar
King Jr TE, Brown KK, Raghu G, du Bois RM, Lynch DA, Martinez F, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184:92–9.
Article
PubMed
Google Scholar
Silver RM. Endothelin and scleroderma lung disease. Rheumatology (Oxford). 2008;47:v25–6.
Article
CAS
Google Scholar