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 J Rare Dis. 2014 Aug 1;9:123. doi: 10.1186/s13023-014-0123-7.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Collagen / biosynthesis*
  • Female
  • Humans
  • Hypertension, Pulmonary / blood
  • Hypertension, Pulmonary / etiology*
  • Lung / cytology
  • Lung / metabolism*
  • Male
  • Muscle, Smooth / cytology
  • Muscle, Smooth / metabolism*
  • Oxidative Stress*
  • Scleroderma, Diffuse / blood
  • Scleroderma, Diffuse / complications*

Substances

  • Collagen