title: Cardiac function in muscular dystrophy associates with abdominal muscle pathology.
abstract: BACKGROUND
  The muscular dystrophies target muscle groups differentially. In mouse models of muscular dystrophy, notably themdxmodel 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, theSgcgmouse, we compared muscle pathology across different muscle groups and heart. A cohort of nearly 200Sgcgmice 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.
  
  
  RESULTS
  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.
authors.affiliation: Molecular Medicine and Pathology, The University of Chicago.
authors.forename: Brandon B
authors.initials: BB
authors.lastname: Gardner
authors.affiliation: Department of Human Genetics, The University of Chicago.
authors.forename: Kayleigh A
authors.initials: KA
authors.lastname: Swaggart
authors.affiliation: Department of Medicine, The University of Chicago.
authors.forename: Gene
authors.initials: G
authors.lastname: Kim
authors.affiliation: Department of Public Health Sciences, Northwestern University.
authors.forename: Sydeaka
authors.initials: S
authors.lastname: Watson
authors.affiliation: Department of Human Genetics, The University of Chicago ; Department of Medicine, The University of Chicago ; The University of Chicago, Center for Genetic Medicine, Northwestern University.
authors.forename: Elizabeth M
authors.initials: EM
authors.lastname: McNally
keywords: abdominal muscle; diaphragm muscle; fibrosis; heart; membrane damage; muscular dystrophy