Increased connective tissue growth factor associated with cardiac fibrosis in the mdx mouse model of dystrophic cardiomyopathy
Carol G Au, Tanya L Butler, Megan C Sherwood, Jonathan R Egan, Kathryn N North, David S Winlaw
INTERNATIONAL JOURNAL OF EXPERIMENTAL PATHOLOGY | WILEY | Published : 2011
Cardiomyopathy contributes to morbidity and mortality in Duchenne muscular dystrophy (DMD), a progressive muscle-wasting disorder. A major feature of the hearts of DMD patients and the mdx mouse model of the disease is cardiac fibrosis. Connective tissue growth factor (CTGF) is involved in the fibrotic process in many organs. This study utilized the mdx mouse model to assess the role of CTGF and other extracellular matrix components during the development of fibrosis in the dystrophic heart. Left ventricular function of mdx and control mice at 6, 29 and 43 weeks was measured by echocardiography. Young (6 weeks old) mdx hearts had normal function and histology. At 29 weeks of age, mdx mice de..View full abstract
Awarded by National Health and Medical Research Council of Australia
C.G.A was supported by a biomedical scholarship (358800) from the National Health and Medical Research Council of Australia. D. S. W was funded by a Career Development Fellowship from the National Heart Foundation of Australia. The authors would like to thank Dr Stephen Twigg (University of Sydney) for his advice on CTGF.