Journal
NEUROMUSCULAR DISORDERS
Volume 18, Issue 5, Pages 371-381Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2008.03.008
Keywords
Duchenne muscular dystrophy; cardiomyopathy; echocardiography; mouse; Nox4; Lox
Categories
Funding
- NHLBI NIH HHS [L40 HL081772-01] Funding Source: Medline
- NICHD NIH HHS [U54 HD053177, 1P30HD40677-01, R24 HD050846-02, 1U54HD053177-01A1, 5R24HD050846-02, R24 HD050846, K12 HD001399, P30 HD040677-019002, U54 HD053177-01A1, P30 HD040677, K12HD001399-04] Funding Source: Medline
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Duchenne muscular dystrophy (DMD; dystrophin-deficiency) causes dilated cardiornyopathy in the second decade of life in affected males. We studied the dystrophin-deficient mouse heart (mdx) using high-frequency echocardiography, histomorphometry, and gene expression profiling. Heart dysfunction was prominent at 9-10 months of age and showed significantly increased LV internal diameter (end systole) and decreased posterior wall thickness. This cardiorryopathy was associated with a 30% decrease in shortening fraction. Histologically, there was a 10-fold increase in connective tissue volume (fibrosis). mRNA profiling with RT-PCR validation showed activation of key pro-fibrotic genes, including Nox4 and Lox. The Nox gene family expression differed in mdx heart and skeletal muscle, where Nox2 was specifically induced in skeletal muscle while Nox4 was specifically induced in heart. This is the first report of an altered profibrotic gene expression profile in cardiac tissue of dystrophic mice showing echocardiographic evidence of cardiomyopathy. (c) 2008 Elsevier B.V. All rights reserved.
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