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 Diseases

Gianfranco Pintus¹  Costanza Emanueli²  Giuseppe Passiu⁴  Gaia Spinetti³  Roberta Giordo¹  Annalisa Cossu¹  Anna Maria Posadino¹  Gian Luca Erre⁴  Francesco Boin⁵ 

[1] Laboratory of Vascular Biology, Department of Biomedical Sciences, University of Sassari, Viale San Pietro 43/B, 07100, Sassari, Italy;Bristol Heart Institute, University of Bristol, Bristol, England, UK;Laboratory of Diabetological Research, IRCCS MultiMedica, Milan, Italy;Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Sassari, Viale San Pietro 8, Sassari, Italy;Johns Hopkins Scleroderma Center, Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Baltimore, MD, USA

关键词: Vascular smooth muscle cells;    Oxidative stress;    Pulmonary arterial hypertension;    Systemic sclerosis;   

Others  :  1150179
DOI  :  10.1186/s13023-014-0123-7

 received in 2014-06-12, accepted in 2014-07-21,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 Boin et al. ; Licensee Biomedcentral Ltd

【 图 表 】

Figure 1.

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