Research on the treatment for xeroderma pigmentosum focusing on the oxidarive DNA damage
Project/Area Number |
24591639
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Dermatology
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Research Institution | Osaka Medical College |
Principal Investigator |
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Project Period (FY) |
2012-04-01 – 2015-03-31
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Project Status |
Completed (Fiscal Year 2014)
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Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2014: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2013: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2012: ¥2,210,000 (Direct Cost: ¥1,700,000、Indirect Cost: ¥510,000)
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Keywords | 色素性乾皮症 / コケイン症候群 / 酸化ストレス / DNA修復 / 抗酸化 / DNA修復 / 紫外線 / 活性酸素 / DNA損傷 / 神経変性 / 皮膚癌 |
Outline of Final Research Achievements |
In the present study, we focused on oxidative DNA damage and its repair in the pathogenesis of neurodegeneration in XP and CS patients. Both XP, CS and XP or CS knockdown cells were hypersensitive to killing by hydrogen peroxide and the level of DNA repair ability to oxidative DNA damage estimated by plasmid host cell reactivation in XP and CS cells was decreased compared to that in normal cells. The hypersensitivity to hydrogen peroxide in XP and CS cells slightly ameliorated after the treatment of some antioxidants. In CS mice, the demyelinating change was observed in the cerebral cortex immunohistochemically, as in MRI image of progressive CS cases. These in vitro and in vivo experimental systems would be a useful tool to explore a novel remedy for the neurologic manifestations.
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Report
(4 results)
Research Products
(31 results)
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[Journal Article] Ocular Manifestations of Xeroderma Pigmentosum: Long-Term Follow-up Highlights the Role of DNA Repair in Protection from Sun Damage.2013
Author(s)
Brooks BP, Thompson AH, Bishop RJ, Clayton JA, Chan CC, Tsilou ET, Zein WM, Tamura D, Khan SG, Ueda T, Boyle J, Oh KS, Imoto K, Inui H, Moriwaki SI, Emmert S, Iliff NT, Bradford P, Digiovanna JJ, Kraemer KH.
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Journal Title
Ophthalmology
Volume: 120
Pages: 1324-1336
Related Report
Peer Reviewed
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[Presentation] UVBと皮膚2014
Author(s)
森脇真一
Organizer
第113回日本皮膚科学会総会
Place of Presentation
京都
Year and Date
2014-06-01
Related Report
Invited
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[Presentation] DNA repair disprders xeroderma pigmentosum abd trichothiodystrophy : benchi to bedside and back2014
Author(s)
Kraemer KH, DiGiovanana JJ, Khan SG, Tamura D, Bradford P, Totonchy M, Goldstein A, Masaki T, Kuschal C, Ueda T, Inui H, Imoto K, Takeuchi S, Moriwaki S
Organizer
International symposium on xeroderma pigmentosum and related diseases : Disorders of DNA damage response ; Bench to Bedside -
Place of Presentation
Kobe, Japan
Related Report
Invited
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