| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2014: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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| Outline of Final Research Achievements |
First, we examined the relationship between TDP-43 and FUS in amyotrophic lateral sclerosis (ALS) using immunohistochemical methods. About 30% of the neurons having TDP-43-positive inclusions lost the nuclear staining of FUS. On the other hand, the nuclear staining of TDP-43 was well preserved in the all neurons containing FUS-positive inclusions. We considered the pathogenic pathway of FUS proteinopathy is independent from TDP-43 proteinopathy, however; abnormal TDP-43 may interact with FUS protein in patients with ALS. Second, we performed neuropathological studies with anti-elongator protein 3 (ELP3) antibodies in patients with ALS. ELP3-positive inclusions were seen in the anterior horn cells, and ELP3 immunoreactivities were co-localized with those of TDP-43. On the other hand, TDP43-positive inclusions in the non-motor neurons were negative for ELP3. Our results suggest that ELP3 may have an important role of the degeneration of motor neurons in ALS.
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