2012 Fiscal Year Final Research Report
Molecular Pathogenesis of Familial Amyotrophic Lateral SclerosisType 6
| Project/Area Number |
22500335
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Neurochemistry/Neuropharmacology
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| Research Institution | Hiroshima Bunkyo Women's University |
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Principal Investigator |
FUJII Ritsuko 広島文教女子大学, 人間科学部, 教授 (90342716)
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| Project Period (FY) |
2010 – 2012
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| Keywords | 精神・神経疾患の病態と治療 |
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| Research Abstract |
Recently, specific point mutations in C-terminus region of TLS have been identified as a cause of familial amyotrophic lateral sclerosis (FALS) type6. TLS with ALS-related point mutations are prone to aggregate and TLS-deficiency is correlated with defective RNA splicing of specific sets of RNAs. Our iCLIP analysis of TLS-deficient mouse brain and mass spectrometry analysis of TLS-protein complex have indicated that TLS-deficiency may induce mitochondrial stress responses and aberrant RNA metabolism in neurons.
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Research Products
(7 results)
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[Journal Article] Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain
Author(s)
B. Rogelj, L.E. Easton, G.K. Bogu, L.W.Stanton, G. Rot, T. Curk, B. Zupan, Y. Sugimoto, M. Modic, N. Haberman, J. Tollervey, R.Fujii, T. Takumi, C E. Shaw and J. Ule
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Journal Title
DOI
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