Budget Amount *help |
¥17,680,000 (Direct Cost: ¥13,600,000、Indirect Cost: ¥4,080,000)
Fiscal Year 2017: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2016: ¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥7,540,000 (Direct Cost: ¥5,800,000、Indirect Cost: ¥1,740,000)
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Outline of Final Research Achievements |
Laser capture microdissection of the mouse spinal motor neurons (SMN) revealed that Rspo2 is highly expressed in spinal motor neurons. Rspo2 induces acetylcholine receptor (AChR) clustering, which is ~80% as potent as agrin. Tissue-specific rescue of Rspo2 by transgenic expression of Rspo2 by SMN- and muscle-specific promoters revealed that SMN-derived Rspo2 plays essential roles in the formation of the neuromuscular junction and AChR clustering. We propose that Rspo2 is an essential AChR clustering-inducing molecule after agrin. We started dissection of molecular pathomechanisms of mutations in a gene encoding glycosylation enzyme, GFPT1, which were identified in Japanese patients with congenital myasthenic syndrome.
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