Study on the therapeutic effect of the chelating agent in Menkes disease by using microPET
| 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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Report
(5 results)
Research Products
(18 results)
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[Journal Article] PET imaging analysis with 64Cu in disulfiram treatment for aberrant copper biodistribution in Menkes disease mouse model.2014
Author(s)
Nomura S, Nozaki S, Hamazaki T, Takeda T, Ninomiya E, Kudo S, Hayashinaka E, Wada Y, Hiroki T, Fujisawa C, Kodama H, Shintaku H, Watanabe Y.
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Journal Title
J Nucl Med
Volume: 55
Issue: 5
Pages: 845-851
DOI
Related Report
Peer Reviewed
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[Presentation] New clinical trial of Autologous Cord Blood Cell Therapy for newborn HIE in Japan.2014
Author(s)
Shintaku H, Nabetani M, Tamura M, Kusuda S, Hayakawa M, Watabe S, Ichiba H, Hamazaki T, Tsuji M, Nagamura T, Taguchi A, Sato Y, Mori R
Organizer
Hot Topics in Neonate 2014
Place of Presentation
Washington DC (USA)
Year and Date
2014-12-07 – 2014-12-11
Related Report
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[Presentation] Effects of two types of copper chelators on copper biodistribution in the kidney of menkes disease model mice by using micropet2014
Author(s)
Shintaku H, Nomura S, Nozaki S, Takeda T, Ninomiya E, Judo S, Hamazaki T, Fujioka H, Hayashinaka E, Wada Y, Fujisawa C, Kodama H, Watanabe Y
Organizer
The3rd Global Congress for Consensus in Pediatrics & Child Health(CIP2014)
Place of Presentation
Millennium Hilton Bangkok Hotel, Thailand
Related Report
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[Presentation] MicroPET imaging is useful to evaluate the effect of disulfiram on copper biodistribution in the kidney of Menkes disease model mice.2013
Author(s)
Nomura S, Nozaki S, Takeda T, Ninomiya E, Judo S, Hamazaki T, Fujioka H, Hayashinaka E, Wada Y, Fujisawa C, Kodama H, Watanabe Y, Shintaku H
Organizer
Society of Nuclear Medicine and Molecular Imaging (SNMMI 2013）
Place of Presentation
Vancouver Convention Center, Canada
Related Report
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