Document Detail

Abnormal cardiac morphology, function and energy metabolism in the dystrophic mdx mouse: an MRI and MRS study.
MedLine Citation:
PMID:  18929569     Owner:  NLM     Status:  MEDLINE    
Patients with muscular dystrophy have abnormal cardiac function and decreased high-energy phosphate metabolism. Here, we have determined whether the 8 month old mdx mouse, an animal model of muscular dystrophy, also has abnormal cardiac function and energetics. In vivo cardiac MRI revealed 33% and 104% larger right ventricular end-diastolic and end-systolic volumes, respectively, and 17% lower right ventricular ejection fractions in mdx mice compared with controls. Evidence of left ventricular diastolic dysfunction included 18% lower peak filling rates in mdx mouse hearts. Abnormal cardiac function was accompanied by necrosis and lower citrate synthase activity in the mdx mouse heart, suggesting decreased mitochondrial content. Decreased mitochondrial numbers were associated with 38% lower phosphocreatine concentration, 22% lower total creatine, 36% higher cytosolic free ADP concentration and 1.3 kJ/mol lower free-energy available from ATP hydrolysis in whole isolated, perfused mdx mouse hearts than in controls. Transsarcolemmal creatine uptake was 12% lower in mdx mouse hearts. We conclude that the absence of dystrophin in adult mdx mouse heart, as in the heart of human patient, is associated with right ventricular dilatation, left ventricular diastolic dysfunction and abnormal energy metabolism.
Wen Zhang; Michiel ten Hove; Jürgen E Schneider; Daniel J Stuckey; Liam Sebag-Montefiore; Britta L Bia; George K Radda; Kay E Davies; Stefan Neubauer; Kieran Clarke
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-09-27
Journal Detail:
Title:  Journal of molecular and cellular cardiology     Volume:  45     ISSN:  1095-8584     ISO Abbreviation:  J. Mol. Cell. Cardiol.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2008-11-17     Completed Date:  2009-01-22     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  0262322     Medline TA:  J Mol Cell Cardiol     Country:  England    
Other Details:
Languages:  eng     Pagination:  754-60     Citation Subset:  IM    
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MeSH Terms
Adenosine Diphosphate / metabolism
Adenosine Triphosphate / metabolism
Citrate (si)-Synthase / metabolism
Energy Metabolism*
Magnetic Resonance Imaging
Mice, Inbred mdx
Mitochondria, Heart / metabolism,  pathology
Muscular Dystrophies / metabolism*,  pathology*,  physiopathology
Muscular Dystrophy, Animal / metabolism*,  pathology*,  physiopathology
Myocardium / metabolism*,  pathology*
Phosphocreatine / metabolism
Sarcolemma / metabolism,  pathology
Stroke Volume
Grant Support
G0600829//Medical Research Council; MC_U137761449//Medical Research Council; PS/02/002/14893//British Heart Foundation; RG/07/004/22659//British Heart Foundation; //British Heart Foundation
Reg. No./Substance:
020IUV4N33/Phosphocreatine; 61D2G4IYVH/Adenosine Diphosphate; 8L70Q75FXE/Adenosine Triphosphate; EC (si)-Synthase

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