Limited impact of fibromodulin deficiency on the development of experimental skin fibrosis

Exp Dermatol. 2016 Jul;25(7):558-61. doi: 10.1111/exd.13012.

Abstract

Excessive production of collagen is the hallmark of fatal diseases of fibrosis such as systemic sclerosis. Overexpression of the proteoglycan fibromodulin (FMOD) has been associated with improved wound healing and scarless repair. In this study, we have investigated the consequences of FMOD deficiency on the development of experimental skin fibrosis. Using immunohistochemistry, we identified FMOD in both human and murine fibrotic skin. In the bleomycin model of skin fibrosis, FMOD(-/-) mice developed skin fibrosis to a similar degree compared to FMOD(+/+) mice. Analysis of skin ultrastructure using transmission electron microscopy revealed a significant reduction in collagen fibril diameter in FMOD(-/-) but not FMOD(+/+) mice following fibrosis. We conclude that the impact of FMOD deficiency on the development of experimental skin fibrosis is limited.

Keywords: bleomycin; collagen; extracellular matrix; skin; systemic sclerosis.

Publication types

  • Letter

MeSH terms

  • Adolescent
  • Animals
  • Bleomycin
  • Case-Control Studies
  • Child
  • Disease Models, Animal
  • Fibromodulin / deficiency*
  • Fibrosis
  • Humans
  • Mice, Inbred C57BL
  • Scleroderma, Systemic / etiology
  • Scleroderma, Systemic / metabolism*
  • Skin / metabolism
  • Skin / pathology
  • Skin / ultrastructure

Substances

  • FMOD protein, human
  • Fmod protein, mouse
  • Bleomycin
  • Fibromodulin