| Budget Amount *help |
¥16,250,000 (Direct Cost: ¥12,500,000、Indirect Cost: ¥3,750,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2012: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2011: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
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| Outline of Final Research Achievements |
Distal myopathy with rimmed vacuoles (DMRV) is one of the protein aggregate myopathies in which numerous proteins and autophagic vacuoles were accumulated in the myofibers. The mechanism, in which the accumulation of these proteins secondarily leads to defects of muscle functions, has not been clarified. In this study, we clarified the following four results: (1) distribution of the accumulated proteins along cytoskeleton, especially microtubules in DMRV myofibers,; (2) no protein accumulation in brain tissues of DMRV model mice; (3) numerous numbers of protein inclusions in atrophic muscles leading to a remarkable weakness in the mice with skeletal muscle-specific deficiency of autophagy as a model of the related disorder; (4) the accumulation of myofibril-regulating proteins, metabolic enzymes and polyubiquitin proteins in the cytoplasmic bodies formed in skeletal muscles in Pompe disease by proteome analysis.
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