Neuropathological and molecular genetic studies of amyotrophic lateral sclerosis
| Project/Area Number |
24591250
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Gunma University |
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Principal Investigator |
FUJITA Yukio 群馬大学, 医学(系)研究科(研究院), 講師 (70420172)
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| Co-Investigator(Kenkyū-buntansha) |
OKAMOTO Koichi 群馬大学, その他部局, 名誉教授 (00124652)
IKEDA Masaki 群馬大学, 医学部附属病院, 講師 (50222899)
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| Project Period (FY) |
2012-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| 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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| Keywords | 筋萎縮性側索硬化症 / ELP3 |
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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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Report
(4 results)
Research Products
(11 results)
