| Budget Amount *help |
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2015: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2014: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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| Outline of Final Research Achievements |
We investigated whether endoplasmic reticulum (ER) stress is essential for motor neuron degeneration in both TDP-43 and FUS/TLS-aggregation models. In motor neurons differentiated from iPS cells derived from ALS patients, some molecules on ER stress pathways were upregulated in mutant FUS/TLS expressing cells. However, only a few molecules were upregulated commonly in all different types of mutant FUS/TLS cells. In TDP-43-aggregation model, it was suggested that sarcoplasmic TDP-43 aggregation induced ER stress and then provided tissue toxicity. In our observations, both FUS/TLS and TDP-43 aggregation might induce ER stress. Therefore, regulation of ER stress pathways would be a therapeutic target for amyotrophic lateral sclerosis.
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