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Analysis of repair function of radiation-induced DNA damage on cultured human fibroblasts derived from patients with xeroderma pigmentosum

Research Project

Project/Area Number 15K09751
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Dermatology
Research InstitutionOsaka Medical College

Principal Investigator

Moriwaki Shinichi  大阪医科大学, 医学部, 教授 (40303565)

Project Period (FY) 2015-04-01 – 2018-03-31
Project Status Completed (Fiscal Year 2017)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2017: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2016: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2015: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
Keywords色素性乾皮症 / 放射線 / DNA二本鎖切断 / γH2AH / 紫外線 / DNA損傷 / H2AX
Outline of Final Research Achievements

Although radiation has been used to examine and sometimes treat patients with xeroderma pigmentosum(XP) and Cockayne syndrome (CS), radiation-induced DNA damage repair function in these patients has not been analyzed in detail. We investigated post-irradiation focus formation using IF staining with gannma-H2AX antibody, a highly sensitive marker of radiation-induced DNA damage (double-strand breaks;DSB) in skin fibroblasts from XP and CS patients. In CSB and XPV (not XPA and XPD/CS) cells, a decrease in focus formation was seen as compared with normal cells.These results suggest that repair of DSB in CS and XPV may be impaired. However, so fat the relation between decrease of DSB repair and XPV and CSB patients' phenotype /genotype was not obvious.

Report

(4 results)
  • 2017 Annual Research Report   Final Research Report ( PDF )
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (17 results)

All 2017 2016 2015

All Journal Article (8 results) (of which Int'l Joint Research: 5 results,  Peer Reviewed: 8 results,  Open Access: 4 results) Presentation (8 results) (of which Int'l Joint Research: 3 results,  Invited: 5 results) Book (1 results)

  • [Journal Article] Lichen planus-like keratosis emerging in a pediatric case of xeroderma pigmentosum group A2017

    • Author(s)
      Terada A, Tanizaki H, Aoshima M, Tokura Y and Moriwaki S
    • Journal Title

      J Dermatol

      Volume: 44 Issue: 7

    • DOI

      10.1111/1346-8138.13883

    • Related Report
      2017 Annual Research Report 2016 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Xeroderma pigmentosum clinical guideline2017

    • Author(s)
      Moriwaki S, Kanda F, Hayashi M, Yamashita D, Sakai Y, Nishigori C
    • Journal Title

      J Dermatol

      Volume: 44 Issue: 10 Pages: 1087-1096

    • DOI

      10.1111/1346-8138.13907

    • Related Report
      2017 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] An XPA gene splicing mutation resulting in trace protein expression in an elderly xeroderma pigmentosum group A patient without neurological abnormalities.2017

    • Author(s)
      Takahashi Y, Endo Y, Kusaka A, Nakamaura S, Nakazawa Y, Ogi T, Uryu M, Tsuji M, Furue M, Moriwaki S.
    • Journal Title

      British Journal of Dermatology

      Volume: 印刷中 Issue: 1 Pages: 253-257

    • DOI

      10.1111/bjd.15051

    • Related Report
      2017 Annual Research Report 2016 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Human DNA repair disorders in dermatology: A historical perspective, current concepts and new insight2016

    • Author(s)
      Moriwaki S
    • Journal Title

      J Derm Sci

      Volume: 81 Issue: 2 Pages: 77-84

    • DOI

      10.1016/j.jdermsci.2015.09.008

    • Related Report
      2016 Research-status Report 2015 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] The present status of xeroderma pigmentosum in Japan and a tentative severity classification scale.2016

    • Author(s)
      Nakano E, Masaki T, Kanda F, Ono R, Takeuchi S, Moriwaki S, Nishigori C.
    • Journal Title

      Exp Dermatol

      Volume: 25 Issue: S3 Pages: 28-33

    • DOI

      10.1111/exd.13082

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Elevated urinary levels of 8-hydroxy-2’-deoxyguanosine in a Japanese child of xeroderma pigmentosum/Cockayne syndrome complex with Infantile onset of nephrotic syndrome2016

    • Author(s)
      Kondo D, Noguchi A, Tamura H, Tsuchida S, Takahashi I, Kubota H, Yano T, Oyama C, Sawaishi Y, Moriwaki S, Takahashi T
    • Journal Title

      Tohoki J Exp Med

      Volume: 239 Issue: 3 Pages: 231-235

    • DOI

      10.1620/tjem.239.231

    • NAID

      130005163073

    • Related Report
      2016 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] Xeroderma pigmentosum complementation group F: report of a case and review of Japanese patients.2015

    • Author(s)
      Tofuku Y, Nobeyama Y, Kamide K, Moriwaki S, Hidemi Nakagawa H
    • Journal Title

      J Dermatol

      Volume: 42 Issue: 10 Pages: 897-9

    • DOI

      10.1111/1346-8138.13013

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] High prevalence of vitamin D deficiency in patients with xeroderma pigmetosum (XP)- A under strict sun-protection2015

    • Author(s)
      Kuwabara A, Tsugawa N, Tanaka K, Uejima Y, Ogawa J, Otao N, Yamada N, Masaki T, Nishigori C, Moriwaki S, Okano T.
    • Journal Title

      European Journal of Clinical Nutrition

      Volume: Epub ahead of print Issue: 6 Pages: 693-9

    • DOI

      10.1038/ejcn.2015.1

    • Related Report
      2015 Research-status Report
    • Peer Reviewed
  • [Presentation] Proposal on the establishment of clinical guidelines for the diagnosis and management of xeroderma pigmentosum and Cockayne syndrome in Japan2016

    • Author(s)
      oriwaki S
    • Organizer
      10th Asian Dermatological Congress
    • Place of Presentation
      Sahara star hotel, Mumbai, India
    • Year and Date
      2016-09-15
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] Decreased repair function of radiation-induced DNA damage on cultured fibroblasts derived from patients with xeroderma pigmentosum variant type2016

    • Author(s)
      Otsuka T, Tanizaki H, Kurokawa T, Nakamura A, Moriwaki S
    • Organizer
      European Society for ermatological Research 46th Annual Meeting
    • Place of Presentation
      Technical University of Munich, Germany
    • Year and Date
      2016-09-07
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] Analysis of acute UVB reaction on basement membrane of the skin using a 3-D cultured human skin model.2016

    • Author(s)
      Hirakawa Y, Tanizaki H, Futaki S, Maemura K, and Moriwaki S
    • Organizer
      Asia-Pacific Combined Dermatology Reaserch Conference 201
    • Place of Presentation
      Sheraton Noosa Resort and Spa, Noosa, Australia
    • Year and Date
      2016-08-25
    • Related Report
      2016 Research-status Report
    • Int'l Joint Research
  • [Presentation] 色素性乾皮症BRUSHU2015

    • Author(s)
      森脇真一
    • Organizer
      第66回日本皮膚科学会中部支部学術大会
    • Place of Presentation
      神戸国際会議場(兵庫県神戸市)
    • Year and Date
      2015-11-01
    • Related Report
      2015 Research-status Report
    • Invited
  • [Presentation] 皮膚科領域の小児慢性特定疾病2015

    • Author(s)
      森脇真一
    • Organizer
      第39回日本小児皮膚科学会
    • Place of Presentation
      かごしま県民交流センター(鹿児島県鹿児島市)
    • Year and Date
      2015-07-25
    • Related Report
      2015 Research-status Report
    • Invited
  • [Presentation] コケイン症候群とトリコチオディストロフィー2015

    • Author(s)
      森脇真一
    • Organizer
      第37回日本光医学光生物学会
    • Place of Presentation
      シーガイヤコンベンションセンター(宮崎県宮崎市)
    • Year and Date
      2015-07-24
    • Related Report
      2015 Research-status Report
    • Invited
  • [Presentation] 日に当たれない小児難病:色素性乾皮症とコケイン症候群~小児慢性特定疾患治療研究事業への期待2015

    • Author(s)
      森脇真一
    • Organizer
      第114回日本皮膚科学会総会
    • Place of Presentation
      パシフィコ横浜(神奈川県横浜市)
    • Year and Date
      2015-05-29
    • Related Report
      2015 Research-status Report
    • Invited
  • [Presentation] 新たな指定難病:色素性乾皮症、コケイン症候群~最近の話題2015

    • Author(s)
      森脇真一
    • Organizer
      第4回光皮膚科学研究会
    • Place of Presentation
      新大阪ワシントンホテル(大阪府大阪市)
    • Year and Date
      2015-05-16
    • Related Report
      2015 Research-status Report
  • [Book] DNA repair disorders --clinical and molecular aspects-2017

    • Author(s)
      Moriwaki S
    • Publisher
      Springer
    • Related Report
      2016 Research-status Report

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Published: 2015-04-16   Modified: 2019-03-29  

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