| Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2014: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
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| Outline of Final Research Achievements |
Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by three cardinal pathological features, including immune abnormalities/inflammation, vasculopathy, and fibrosis. Although the pathogenesis of SSc still remains unknown, it is generally accepted that environmental factors play a critical role in the development of this disease in genetically predisposed individuals. In SSc dermal fibroblasts, two transcription factors, Fli1 and KLF5, are epigenetically suppressed, suggesting that the downregulation of these molecules is likely to be predisposing factors of SSc. Of note, mice with heterozygous deletion of Fli1 and Klf5 spontaneously developed the three cardinal pathological features of SSc, strictly recapitulating the sequential pathological process (initiating with immune abnormalities, followed by vasculopathy, and eventually resulting in tissue fibrosis). Further studies with this murine model would provide a useful clue to understand SSc pathogenesis.
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