Understanding the origin, activation and regulation of matrix-producing myofibroblasts for treatment of fibrotic disease

Rafael Kramann, Derek P. Dirocco, Benjamin D. Humphreys

Research output: Contribution to journalArticlepeer-review

155 Scopus citations

Abstract

Fibrosis and scar formation results from chronic progressive injury in virtually every tissue and affects a growing number of people around the world. Myofibroblasts drive fibrosis, and recent work has demonstrated that mesenchymal cells, including pericytes and perivascular fibroblasts, are their main progenitors. Understanding the cellular mechanisms of pericyte/fibroblast-to- myofibroblast transition, myofibroblast proliferation and the key signalling pathways that regulate these processes is essential to develop novel targeted therapeutics for the growing patient population suffering from solid organ fibrosis. In this review, we summarize the current knowledge about different progenitor cells of myofibroblasts, discuss major pathways that regulate their transdifferentiation and discuss the current status of novel targeted anti-fibrotic therapeutics in development.

Original languageEnglish
Pages (from-to)273-289
Number of pages17
JournalJournal of Pathology
Volume231
Issue number3
DOIs
StatePublished - Nov 2013

Keywords

  • fibrosis
  • interstitium
  • myofibroblast
  • pericyte

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