Using the Bleomycin-Induced Model of Fibrosis to Study the Contribution of CCN Proteins to Scleroderma Fibrosis

Methods Mol Biol. 2023:2582:309-321. doi: 10.1007/978-1-0716-2744-0_21.

Abstract

Approximately 45% of the deaths in the developed world result from conditions with a fibrotic component. Although no specific, focused anti-fibrotic therapies have been approved for clinical use, a long-standing concept is that targeting CCN proteins may be useful to treat fibrosis. Herein, we summarize current data supporting the concept that targeting CCN2 may be a viable anti-fibrotic approach to treat scleroderma. Testing this hypothesis has been made possible by using a mouse model of inflammation-driven skin and lung fibrosis.

Keywords: Bleomycin; CCN proteins; CCN2; CTGF; Fibrosis; Lung fibrosis; Matricellular proteins; Scleroderma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Bleomycin / adverse effects
  • Connective Tissue Growth Factor / genetics
  • Connective Tissue Growth Factor / metabolism
  • Disease Models, Animal
  • Fibrosis
  • Pulmonary Fibrosis* / chemically induced
  • Pulmonary Fibrosis* / metabolism
  • Scleroderma, Systemic* / chemically induced
  • Scleroderma, Systemic* / metabolism
  • Skin / metabolism

Substances

  • Bleomycin
  • Connective Tissue Growth Factor

Grants and funding