Increased Connective Tissue Growth Factor Expression Associated with Functional Impairment and Extracellular Matrix Remodelling in Dystrophin-Deficient Heart

Introduction: Cardiomyopathy is a major cause of morbidity and mortality in Duchenne muscular dystrophy (DMD), a progressive X-linked neuromuscular disease caused by dystrophin gene mutations. DMD hearts are characterised by extracellular matrix (ECM) expansion and fibrosis. We examined the role of connective tissue growth factor (CTGF) and transforming growth factor (TGF)-β in cardiac fibrosis and dysfunction in the dystrophin-deficient mdx mouse model of DMD.