Boin, Francesco and Erre, Gian Luca and Posadino, Anna Maria and Cossu, Annalisa and Giordo, Roberta and Spinetti, Gaia and Passiu, Giuseppe and Emanueli, Costanza and Pintus, Gianfranco (2014) Oxidative stress-dependent activation of collagen synthesis is induced in human pulmonary smooth muscle cells by sera from patients with scleroderma-associated pulmonary hypertension. Orphanet Journal of Rare Deseases, Vol. 9 (123), p. 5. ISSN 1750-1172. Article.
Pulmonary arterial hypertension is a major complication of systemic sclerosis. Although oxidative stress, intima hyperplasia and a progressive vessel occlusion appear to be clearly involved, the fine molecular mechanisms underpinning the onset and progression of systemic sclerosis-associated pulmonary arterial hypertension remain largely unknown. Here we shows for the first time that an increase of NADPH-derived reactive oxygen species production induced by sera from systemic sclerosis patients with pulmonary arterial hypertension drives collagen type I promoter activity in primary human pulmonary artery smooth muscle cells, suggesting that antioxidant-based therapies should be considered in the treatment of systemic sclerosis-associated vascular diseases.
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