Horvath, Deanna Maree (2011) The effect of taurine on dystrophic muscle tissue function. PhD thesis, Victoria University.

Abstract

Duchenne muscular dystrophy (DMD) is a lethal X-linked genetic disorder which results in chronic degeneration of skeletal muscle, significantly impacting on the duration and quality of life. Despite the genetic defect and the missing protein dystrophin having been identified and characterised over 20 years ago, curative genetic therapies are still not clinically applicable, and corticosteroids, which are the only significantly beneficial treatment option currently available to DMD patients, are associated with several side-effects. Thus, there is a need for additional therapeutic interventions that can improve skeletal muscle function and delay the onset of severe pathology in dystrophy. The amino acid taurine is essential for normal skeletal muscle function, and has been shown to act on several factors thought to be key contributors to the development of skeletal muscle pathology in dystrophy. Moreover, as dystrophic skeletal muscle demonstrates a significant decrease in taurine content, it is possible that raising intramuscular taurine stores may preserve muscle function in dystrophy, and thus have potential therapeutic applications. Despite this, only two studies have ever examined the effect of taurine supplementation on dystrophic muscle function. The purpose of this thesis was to examine the effect of taurine on dystrophic skeletal muscle function, which was performed in three studies using the dystrophic mdx mouse as a model for DMD.

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