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

    Research output: ThesisDoctoral Thesis

    Abstract

    [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.
    LanguageEnglish
    QualificationDoctor of Philosophy
    StateUnpublished - 2010

    Fingerprint

    Inbred mdx Mouse
    Dystrophin
    Duchenne Muscular Dystrophy
    Necrosis
    Wasting Syndrome
    Muscle Weakness
    Life Expectancy
    Connective Tissue
    Regeneration
    Skeletal Muscle
    Proteins
    Oxidative Stress
    Therapeutics
    Inflammation
    Calcium
    Muscles
    Membranes

    Cite this

    @phdthesis{1c5e00b55c2e4023b297663a84d0c853,
    title = "Therapeutic interventions for Duchenne Muscular Dystrophy - studies in the mdx mouse",
    abstract = "[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.",
    keywords = "Duchene muscular dystrophy, Mdx mouse, Exercise, Therapeutic interventions, Anti-inflammatory, Skeletal muscle",
    author = "Hannah Crabb",
    year = "2010",
    language = "English",

    }

    TY - THES

    T1 - Therapeutic interventions for Duchenne Muscular Dystrophy - studies in the mdx mouse

    AU - Crabb,Hannah

    PY - 2010

    Y1 - 2010

    N2 - [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.

    AB - [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.

    KW - Duchene muscular dystrophy

    KW - Mdx mouse

    KW - Exercise

    KW - Therapeutic interventions

    KW - Anti-inflammatory

    KW - Skeletal muscle

    M3 - Doctoral Thesis

    ER -