Development of phenotype predictive diagnosis method and treatment in adrenoleukodystrophy by the methods combines patient resource and disease model
| Project/Area Number |
15H04875
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Gifu University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
TAKASHIMA Shigeo 岐阜大学, 生命科学総合研究支援センター, 助教 (50537610)
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥16,900,000 (Direct Cost: ¥13,000,000、Indirect Cost: ¥3,900,000)
Fiscal Year 2017: ¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2016: ¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2015: ¥8,580,000 (Direct Cost: ¥6,600,000、Indirect Cost: ¥1,980,000)
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| Keywords | 副腎白質ジストロフィー / 脱髄 / 造血幹細胞移植 / 早期診断 / 病型規定因子 / 神経接着因子 / マイクロアレイ解析 / 二頭酵素欠損症 / 神経細胞接着因子 / 大脳型発症因子 / 疾患モデルマウス / 極長鎖脂肪酸 / ペルオキシソーム / 造血細胞移植 / モデル生物 / iPS細胞 / モデルマウス |
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| Outline of Final Research Achievements |
Achievement of early diagnosis system by academia: 77 cases of ALD patients were diagnosed and the latest medical information was provided. Among them, cerebral type patients as transplant indication were provided in the shortest 2 days, leading to improvement of patient prognosis from early transplantation.
Neuronal cell adhesion factor was specified as cerebral-type related candidate genes by microarray analysis of RNA samples from the patients between before and after transplantation. Even in gene expression analysis with further cases, variation of the factor was verified. Meanwhile, there was no clear difference of the factor between patient samples from cerebral type and those from non-cerebral type AMN. As a result of gene expression analysis of the liver by administering Lorenzo’s Oil to mice, it became clear that gene expression of peroxisomal D-bifunctional protein (DBP) was increased. We will also conduct research on the clinical application to DBP deficient patients.
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Report
(4 results)
Research Products
(22 results)
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[Journal Article] Profiling and Imaging of Phospholipids in Brains of Abcd1-Deficient Mice.2018
Author(s)
Hama K, Fujiwara Y, Morita M, Yamazaki F, Nakashima Y, Takei S, Takashima S, Setou M, Shimozawa N, Imanaka T, Yokoyama K.
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Journal Title
Lipids
Volume: 53
Issue: 1
Pages: 85-102
DOI
Related Report
Peer Reviewed / Open Access
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