Skeletal muscle fibrosis develops in response to desmin deletion

Gretchen A. Meyer, Richard L. Lieber

Research output: Contribution to journalArticlepeer-review

35 Scopus citations

Abstract

Skeletal muscle is a dynamic composite of proteins that responds to both internal and external cues to facilitate muscle adaptation. In cases of disease or altered use, these messages can be distorted resulting in myopathic conditions such as fibrosis. In this work, we describe a mild and progressive fibrotic adaptation in skeletal muscle lacking the cytoskeletal intermediate filament protein desmin. Muscles lacking desmin become progressively stiffer, accumulate increased collagen, and increase expression of genes involved in extracellular matrix turnover. Additionally, in the absence of desmin, skeletal muscle is in an increased state of inflammation and regeneration as indicated by increased centrally nucleated fibers, elevated inflammation and regeneration related gene expression, and increased numbers of inflammatory cells. These data suggest a potential link between increased cellular damage and the development of fibrosis in muscles lacking the cytoskeletal support of the desmin filament network.

Original languageEnglish (US)
Pages (from-to)1609-1620
Number of pages12
JournalAmerican Journal of Physiology - Cell Physiology
Volume302
Issue number11
DOIs
StatePublished - Jun 1 2012

Keywords

  • Adaptation
  • Extracellular matrix

ASJC Scopus subject areas

  • Physiology
  • Cell Biology

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