| Budget Amount *help |
¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000)
Fiscal Year 2013: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
Fiscal Year 2012: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
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| Research Abstract |
Fibrodysplasia ossificans progressiva (FOP) is a severely disabling heritable disorder of connective tissue characterized by progressive extraskeletal ossification in skeletal muscle. Mutation of the ALK-2 gene, a BMP type I receptor, was identified in FOP patients and has been shown to contribute to the pathogenesis of FOP. Skeletal muscle contains two types of progenitor cells: satellite cells and mesenchymal progenitor cells (MPC). In this study, we investigated possible involvement of these progenitors in the pathogenesis of FOP. We established techniques for isolating and culturing satellite cells and MPC from human skeletal muscle. Human MPC showed much higher osteogenic potential than human satellite cells and MPC transduced with mutant ALK-2 contributed heterotopic ossification when transplanted into immunodeficient mice. Our results strongly suggest that MPC represents best possible candidate for cellular origin of heterotopic ossification in skeletal muscle.
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