title: The protective effect of hydrogen sulfide on systemic sclerosis associated skin and lung fibrosis in mice model.
abstract: BACKGROUND
  Systemic sclerosis (SSc) caused fibrosis can be fatal and it still lack of effective treatment. Hydrogen sulfide (H2S) appears to be an attractive therapeutic candidates. This study aimed to investigate the protective effect of H2S on SSc-associated skin and lung fibrosis.
  
  
  METHODS
  We developed a model of SSc by subcutaneous injecting BLM to female C3H mice. The mice received daily subcutaneous injections of NaHS (56 and 112 μg/kg), an H2S donor. On days 7, 28, and 42, the mice were killed and blood samples were collected to measure the plasma H2S concentration, the skin and lung tissues was harvested for microscopic examination, immunohistochemistry and quantify biological parameters (hydroxyproline content, RT-qPCR and Western blot).
  
  
  RESULTS
  In model group, the dermis of skin tissues at different time points gradually thickened, collagen deposition increased. The lung tissues presented pathological changes such as obvious inflammatory cell infiltration, increased collagen deposition and the plasma H2S concentrations points significantly decreased. Administration of NaHS markedly decreased the biomarkers of fibrosis such as α-smooth muscle actin, collagen-I, collagen-III, fibronectin, transforming growth factor-β1, Smad2/3 phosphorylation and inflammation including the marker protein of monocyte/macrophage and monocyte chemoattractant protein-1 in the lung. Compared to the low dose group, the expression in the high dose group have decreased trend, but the difference was not significant.
  
  
  CONCLUSIONS
  We demonstrate the beneficial effects of H2S on SSc-associated skin and lung fibrosis. H2S may be a potential therapy against this intractable disease.
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Zhi
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Xiaoya
authors.initials: X
authors.lastname: Yin
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Luyan
authors.initials: L
authors.lastname: Gao
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Sheng
authors.initials: S
authors.lastname: Feng
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Kai
authors.initials: K
authors.lastname: Song
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Lingyun
authors.initials: L
authors.lastname: Li
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Department of Nephrology, Second Affiliated Hospital of Soochow University, 1055 Sanxiang Road, Jinchang, Suzhou, 215000 Jiangsu Province China.
authors.forename: Huaying
authors.initials: H
authors.lastname: Shen
keywords: Fibrosis; Hydrogen sulfide; Systemic sclerosis; Transforming growth factor-β1