| 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)
|
|---|
| Outline of Final Research Achievements |
This study was conducted to clarify ubiquilin 2, a novel amyotrophic lateral sclerosis (ALS)-related protein that the research manager helped to identify, and p62 of which mutations in the first Japanese patients were found by the manager. We used pluripotent stem (iPS) cells of patients with ALS and controls. We found neurons derived from iPS cells of a patient with a p62 mutation had aggregations of p62, a pathological hallmark of ALS. However, the aggregates were not positive for ubiquilin 2 or valosin-containing protein (VCP). Control neurons were negative for p62. Cells of a p62-negative patient had mild aggregations. Treatment of an autophagy-inducer and an antioxidant reduced aggregation.
|
