Cardiac function in muscular dystrophy associates with abdominal muscle pathology

Brandon B. Gardner, Kayleigh A. Swaggart, Gene Kim, Sydeaka Watson, Elizabeth M. McNally*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Background: The muscular dystrophies target muscle groups differentially. In mouse models of muscular dystrophy, notably the mdx model of Duchenne Muscular Dystrophy, the diaphragm muscle shows marked fibrosis and at an earlier age than other muscle groups, more reflective of the histopathology seen in human muscular dystrophy. Methods: Using a mouse model of limb girdle muscular dystrophy, the Sgcg mouse, we compared muscle pathology across different muscle groups and heart.Acohort of nearly 200 Sgcg mice were studied using multiple measures of pathology including echocardiography, Evans Blue dye uptake and hydroxyproline content in multiple muscle groups. Spearman rank correlations were determined among echocardiographic and pathological parameters. Findings: The abdominal muscles were found to have more fibrosis than other muscle groups, including the diaphragm muscle. The abdominal muscles also had more Evans blue dye uptake than other muscle groups. The amount of diaphragm fibrosis was found to correlate positively with fibrosis in the left ventricle, and abdominal muscle fibrosis correlated with impaired left ventricular function. Fibrosis in the abdominal muscles negatively correlated with fibrosis in the diaphragm and right ventricles. Together these data reflect the recruitment of abdominal muscles as respiratory muscles in muscular dystrophy, a finding consistent with data from human patients.

Original languageEnglish (US)
Pages (from-to)39-49
Number of pages11
JournalJournal of neuromuscular diseases
Volume2
Issue number1
DOIs
StatePublished - 2015

Keywords

  • Abdominal muscle
  • Diaphragm muscle
  • Fibrosis
  • Heart
  • Membrane damage
  • Muscular dystrophy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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