Mouse Chimeras and Genetic Rescue of Mosaic Muscle
The precise developmental mechanisms leading to myoblast fusion and the subsequent functional relationships that exist amongst the myonuclei contained within each syncytium are for the most part ill-defined. In contrast, extraordinary advances have been achieved in defining the precise molecular deficits causing several inherited diseases of muscle. In such diseases, functional rescue of muscle might be achievable by the introduction of genetically normal myonuclei but this strategy ultimately depends upon the interrelationships that exist between the myonuclei and the syncytial cytoplasm. If the mRNA encoded by each myonucleus was translated into protein molecules that were subsequently restricted to a “nuclear territory”, rescue of such mosaic fibers might depend upon not only the number of transplanted myonuclei but also their intrafiber distribution. Alternatively, if proteins encoded by each myonucleus were distributed uniformly throughout the syncytium, the spatial organization of the transplanted myonuclei could be irrelevant.
KeywordsSchwann Cell Muscular Dystrophy Myoblast Fusion Dystrophin Deficiency Dystrophia Muscularis
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