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Casey McCarthy

Duchenne Muscular Dystrophy y Occurs due to a fault in genes y Only occurs in males X linked y Affects both skeletal and cardiac muscles y Progressive and lethal y Currently no cure .

Multipotential mesoangioblast stem cell therapy y Stem cells y Previous research has linked mesoangioblasts with research into other types of muscular dystrophy y Undifferentiated y Lineage can be either endothelial .

Mouse Model y Contain a mutation in the dystrophin gene y Double knockout mice utrophin y Severe muscle disease.MDX/UTRN-/. y Progressive fibre degeneration and fibrosis y Centrally localised nuclei in skeletal muscle y Change in ratio of fibre types y Joint contractures y Cardiomyopathy y Kyphosis .

Objective .

Materials & Methods y Utrophin & dystrophin deficient double knockout mice y Isolation of cells from aorta y Cell culture y Labelling cells with fluorescent tracers y In vitro differentation y Oil red O staining y Intramuscular injection of mesioangioblasts and tissue preperation for analysis .

y Hematoxylin & eosin staining y BrdU labelling of proliferating cells y Western blotting y Indirect Immunofluoresence y Analysis of Evans blue die uptake in tissue .

utrn gastrocnemius muscle y Mesoangioblasts contribute to the satellite cell population .Results y Isolation of mesoangioblasts y Mesoangioblasts differntiate into skeletal muscle in vivo y Mesoangioblasts proliferate in vivo y Mesoangioblasts restore dystrophin and utrophin in mdx/utrn gastrocnemius y Mesoangioblasts decreases membrane damage in mdx.

Discussion y Stem cells can repair and regenerate skeletal muscle y Mesoangioblast cells produce fibers which are protected from further damage y Mesoangioblasts contribute to repair for up to 9 weeks after injection y These stem cells can be used to prevent disease progression in muscular dystrophies .