2013 Fiscal Year Final Research Report
A gain of toxic function hypothesis via accumulated RNA-binding protein and repeat RNA in the pathogenesis of ALS and its validation in vivo
| Project/Area Number |
24659438
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | 独立行政法人国立精神・神経医療研究センター |
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Principal Investigator |
NAGAI Yoshitaka 独立行政法人国立精神・神経医療研究センター, 神経研究所疾病研究第四部, 室長 (60335354)
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| Project Period (FY) |
2012-04-01 – 2014-03-31
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| Keywords | 遺伝子 / 神経科学 / 蛋白質 / 脳神経疾患 / RNA / ALS / リピートRNA / RNA結合蛋白質 |
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| Research Abstract |
Based on the hypothesis that abnormal accumulation of RNA would trigger aggregation and accumulation of the RNA-binding proteins, eventually leading to neurodegeneration in the pathogenesis of amyotrophic lateral sclerosis (ALS), 1) we established novel ALS model flies expressing expanded GGGGCC repeat RNA, which mimic neurological phenotypes and pathology of human ALS patients. 2) Furthermore, using this newly-established ALS models flies, we have successfully identified some RNA-binding proteins that can modify the toxicity of expanded GGGGCC repeat RNA in vivo. We therefore conclude that RNA-binding proteins are involved in neurotoxicity of abnormal RNA accumulation, which can contribute to the pathomechanism of ALS.
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Research Products
(33 results)
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[Journal Article] Identification of ter94, Drosophila VCP, as a strong modulator of motor neuron degeneration induced by knockdown of Caz, Drosophila FUS2014
Author(s)
Azuma Y., Tokuda T., Shimamura M., Kyotani A., Sasayama H., Yoshida T., Mizuta I., Mizuno T., Nakagawa M., Fujikake N., Ueyama M., Nagai Y., Yamaguchi M.
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Journal Title
Hum. Mol. Genet
Volume: 23(13)
Pages: 3467-3480
DOI
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