| Project/Area Number |
24591523
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Osaka City University |
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Principal Investigator |
SHINTAKU HARUO 大阪市立大学, 大学院医学研究科, 教授 (00206319)
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| Co-Investigator(Kenkyū-buntansha) |
SHIOMI Susumu 大阪市立大学, 大学院医学研究科, 教授 (30170848)
HAMAZAKI Takashi 大阪市立大学, 大学院医学研究科, 講師 (40619798)
FUJIOKA Hiroki 大阪市立大学, 大学院医学研究科, 客員研究員 (70382083)
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| Project Period (FY) |
2012-04-01 – 2016-03-31
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| Project Status |
Completed (Fiscal Year 2015)
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| Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2014: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2013: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2012: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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| Keywords | メンケス病 / 銅 / ATP7A / ジスルフィラム / ペニシラミン / マイクロペット / キレート剤 / 発達遅滞 / 発達遅延 / microPET / dペニシラミン / ｄペニシラミン |
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| Outline of Final Research Achievements |
Menkes disease is a congenital copper metabolisc disorder, leading to both copper deficiency in brain and copper accumulation in the kidney due to copper transport disorder. Macula mice is used as Menkes disease model. Pretreatment with the lipophilic chelator disulfiram markedly increased copper uptake to the brain of macular mice. Pretreatment with the water-soluble chelator penicillamine enhanced urinary excretion and reduced copper accumulation in the kidney. Concomitant treatment with disulfiram and penicillamine retained the same effects. Combined use of disulfiram and penicillamine may improve clinical outcomes of Menkes disease. Therefore, it is possible to improve the neurologic outcome of Menkes disease, further revealed the possibility of improving the long-term prognosis of renal function.
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