Oxidative stress-dependent activation of collagen synthesis is induced in human pulmonary smooth muscle cells by sera from patients with scleroderma-associated pulmonary hypertension

Francesco Boin, Gian Luca Erre, Anna Maria Posadino, Annalisa Cossu, Roberta Giordo, Gaia Spinetti, Giuseppe Passiu, Costanza Emanueli, Gianfranco Pintus

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Article number123
JournalOrphanet journal of rare diseases
Volume9
Issue number1
DOIs
StatePublished - Aug 1 2014

Keywords

  • Oxidative stress
  • Pulmonary arterial hypertension
  • Systemic sclerosis
  • Vascular smooth muscle cells

ASJC Scopus subject areas

  • Genetics(clinical)
  • Pharmacology (medical)

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