Animal models of scleroderma: Recent progress

Roberta Goncalves Marangoni*, John Varga, Warren Tourtellotte

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

17 Scopus citations

Abstract

Purpose of review We discuss recent advances in evaluating and optimizing animal models of systemic sclerosis (SSc). Such models could be of value for illuminating etiopathogenesis using hypothesis-testing experimental approaches, for developing effective disease-modifying therapies, and for uncovering clinically relevant biomarkers. Recent findings We describe recent advances in previously reported and novel animal models of SSc. The limitations of each animal model and their ability to recapitulate the pathophysiology of recognized molecular subsets of SSc are discussed. We highlight attrition of dermal white adipose tissue as a consistent pathological feature of dermal fibrosis in mouse models, and its relevance to SSc-associated cutaneous fibrosis. Summary Several animal models potentially useful for studying SSc pathogenesis have been described. Recent studies highlight particular strengths and weaknesses of selected models in recapitulating distinct features of the human disease. When used in the appropriate experimental setting, and in combination, these models singly and together provide a powerful set of in-vivo tools to define underlying mechanisms of disease and to develop and evaluate effective antifibrotic therapies.

Original languageEnglish (US)
Pages (from-to)561-570
Number of pages10
JournalCurrent Opinion in Rheumatology
Volume28
Issue number6
DOIs
StatePublished - Oct 1 2016

Keywords

  • Animal models
  • Gene targeting
  • Scleroderma
  • Systemic sclerosis
  • Transgenic mice

ASJC Scopus subject areas

  • Rheumatology

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