Therapeutic interventions for Duchenne Muscular Dystrophy - studies in the mdx mouse

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[Truncated abstract] Duchenne Muscular Dystrophy (DMD) is a lethal X-linked muscle wasting disease resulting from defects in the myofibre subsarcolemmal protein dystrophin. The lack of functional dystrophin protein leads to myofibre membrane fragility, repeated cycles of myofibre necrosis and regeneration, and the eventual replacement of skeletal muscle by fatty and fibrous connective tissue. DMD is characterised by progressive muscle weakness and wasting with a limited life expectancy of approximately 20 years in humans. Myofibre necrosis is the fundamental destructive process in DMD although the exact mechanism by which the absence of dystrophin leads to myofibre necrosis is unknown; inflammation, oxidative stress, metabolic abnormality, mislocalisation of nNOS and increased intracellular calcium are all heavily implicated in the process.
Original languageEnglish
QualificationDoctor of Philosophy
StateUnpublished - 2010

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