| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Outline of Final Research Achievements |
FUS/TLS is an RNA-binding protein associated with neurodegenerative diseases. Mutations of FUS/TLS are causative of amyotrophic lateral sclerosis (ALS), while FUS/TLS protein is accumulated in the inclusion bodies of a subset of frontotemporal lobar degeneration and polyglutamine diseases. However, pathological roles of FUS/TLS in these diseases have been elusive. We made homozygous FUS/TLS knockout mice, which showed some behavioral alterations but did not manifest ALS-like phenotypes. We then crossed TLS heterozygous mice with Huntington’s disease model mice and found that FUS/TLS heterozygosity worsened the HD phenotypes. In conclusion, our results indicate that loss of FUS/TLS function is not sufficient for causing ALS, while reduced function of FUS/TLS can modify the disease severity of HD.
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