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Inactivation of urokinase-type plasminogen activator receptor (uPAR) gene induces dermal and pulmonary fibrosis and peripheral microvasculopathy in mice: a new model of experimental scleroderma?

Manetti M, Rosa I, Milia AF, Guiducci S, Carmeliet P, Ibba-Manneschi L, Matucci-Cerinic M.

Ann Rheum Dis. 2014 Sep;73(9):1700-9. doi: 10.1136/annrheumdis-2013-203706. Epub 2013 Jul 12.


Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis.

Manetti M, Romano E, Rosa I, Guiducci S, Bellando-Randone S, De Paulis A, Ibba-Manneschi L, Matucci-Cerinic M.

Ann Rheum Dis. 2017 May;76(5):924-934. doi: 10.1136/annrheumdis-2016-210229. Epub 2017 Jan 6.


Upregulation of the N-formyl Peptide receptors in scleroderma fibroblasts fosters the switch to myofibroblasts.

Rossi FW, Napolitano F, Pesapane A, Mascolo M, Staibano S, Matucci-Cerinic M, Guiducci S, Ragno P, di Spigna G, Postiglione L, Marone G, Montuori N, de Paulis A.

J Immunol. 2015 Jun 1;194(11):5161-73. doi: 10.4049/jimmunol.1402819. Epub 2015 Apr 27.


The absence of uPAR is associated with the progression of dermal fibrosis.

Kanno Y, Kaneiwa A, Minamida M, Kanno M, Tomogane K, Takeuchi K, Okada K, Ueshima S, Matsuo O, Matsuno H.

J Invest Dermatol. 2008 Dec;128(12):2792-7. doi: 10.1038/jid.2008.157. Epub 2008 Jun 12. Erratum in: J Invest Dermatol. 2009 Jul; 129(7):1838.


Critical role of the adhesion receptor DNAX accessory molecule-1 (DNAM-1) in the development of inflammation-driven dermal fibrosis in a mouse model of systemic sclerosis.

Avouac J, Elhai M, Tomcik M, Ruiz B, Friese M, Piedavent M, Colonna M, Bernhardt G, Kahan A, Chiocchia G, Distler JH, Allanore Y.

Ann Rheum Dis. 2013 Jun;72(6):1089-98. doi: 10.1136/annrheumdis-2012-201759. Epub 2012 Nov 17.


Transcription factor fos-related antigen-2 induces progressive peripheral vasculopathy in mice closely resembling human systemic sclerosis.

Maurer B, Busch N, Jüngel A, Pileckyte M, Gay RE, Michel BA, Schett G, Gay S, Distler J, Distler O.

Circulation. 2009 Dec 8;120(23):2367-76. doi: 10.1161/CIRCULATIONAHA.109.855114. Epub 2009 Nov 23.


CD109 overexpression ameliorates skin fibrosis in a mouse model of bleomycin-induced scleroderma.

Vorstenbosch J, Al-Ajmi H, Winocour S, Trzeciak A, Lessard L, Philip A.

Arthritis Rheum. 2013 May;65(5):1378-83. doi: 10.1002/art.37907.


Increased serum levels and tissue expression of matrix metalloproteinase-12 in patients with systemic sclerosis: correlation with severity of skin and pulmonary fibrosis and vascular damage.

Manetti M, Guiducci S, Romano E, Bellando-Randone S, Conforti ML, Ibba-Manneschi L, Matucci-Cerinic M.

Ann Rheum Dis. 2012 Jun;71(6):1064-72. doi: 10.1136/annrheumdis-2011-200837. Epub 2012 Jan 17.


Increased plasma soluble urokinase plasminogen activator receptor levels in systemic sclerosis: possible association with microvascular abnormalities and extent of fibrosis.

Legány N, Toldi G, Distler JH, Beyer C, Szalay B, Kovács L, Vásárhelyi B, Balog A.

Clin Chem Lab Med. 2015 Oct;53(11):1799-805. doi: 10.1515/cclm-2015-0079.


Recent advances in animal models of systemic sclerosis.

Asano Y.

J Dermatol. 2016 Jan;43(1):19-28. doi: 10.1111/1346-8138.13185. Review.


Postnatal induction of transforming growth factor beta signaling in fibroblasts of mice recapitulates clinical, histologic, and biochemical features of scleroderma.

Sonnylal S, Denton CP, Zheng B, Keene DR, He R, Adams HP, Vanpelt CS, Geng YJ, Deng JM, Behringer RR, de Crombrugghe B.

Arthritis Rheum. 2007 Jan;56(1):334-44.


The transcription factor Fra-2 regulates the production of extracellular matrix in systemic sclerosis.

Reich N, Maurer B, Akhmetshina A, Venalis P, Dees C, Zerr P, Palumbo K, Zwerina J, Nevskaya T, Gay S, Distler O, Schett G, Distler JH.

Arthritis Rheum. 2010 Jan;62(1):280-90. doi: 10.1002/art.25056.


Identification of cadherin 11 as a mediator of dermal fibrosis and possible role in systemic sclerosis.

Wu M, Pedroza M, Lafyatis R, George AT, Mayes MD, Assassi S, Tan FK, Brenner MB, Agarwal SK.

Arthritis Rheumatol. 2014 Apr;66(4):1010-21. doi: 10.1002/art.38275.


Downregulation of miR-193b in systemic sclerosis regulates the proliferative vasculopathy by urokinase-type plasminogen activator expression.

Iwamoto N, Vettori S, Maurer B, Brock M, Pachera E, Jüngel A, Calcagni M, Gay RE, Whitfield ML, Distler JH, Gay S, Distler O.

Ann Rheum Dis. 2016 Jan;75(1):303-10. doi: 10.1136/annrheumdis-2014-205326. Epub 2014 Nov 10.


Insulin-like growth factor binding protein 5 induces skin fibrosis: A novel murine model for dermal fibrosis.

Yasuoka H, Jukic DM, Zhou Z, Choi AM, Feghali-Bostwick CA.

Arthritis Rheum. 2006 Sep;54(9):3001-10.


The inflammasome activating caspase 1 mediates fibrosis and myofibroblast differentiation in systemic sclerosis.

Artlett CM, Sassi-Gaha S, Rieger JL, Boesteanu AC, Feghali-Bostwick CA, Katsikis PD.

Arthritis Rheum. 2011 Nov;63(11):3563-74. doi: 10.1002/art.30568.


Systemic sclerosis fibroblasts inhibit in vitro angiogenesis by MMP-12-dependent cleavage of the endothelial cell urokinase receptor.

Serratì S, Cinelli M, Margheri F, Guiducci S, Del Rosso A, Pucci M, Fibbi G, Bazzichi L, Bombardieri S, Matucci-Cerinic M, Del Rosso M.

J Pathol. 2006 Oct;210(2):240-8.


Myofibroblast induction and microvascular alteration in scleroderma lung fibrosis.

Beon M, Harley RA, Wessels A, Silver RM, Ludwicka-Bradley A.

Clin Exp Rheumatol. 2004 Nov-Dec;22(6):733-42.


Antifibrotic effects of crocetin in scleroderma fibroblasts and in bleomycin-induced sclerotic mice.

Song Y, Zhu L, Li M.

Clinics (Sao Paulo). 2013 Oct;68(10):1350-7. doi: 10.6061/clinics/2013(10)10.

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