Treatment and elucidation of generation mechanism of the liver trouble in Wilson' s disease

• Project/Area Number: 19790743
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Single-year Grants
• Research Field: Pediatrics
• Research Institution: Teikyo University
• Principal Investigator: FUJISAWA Chie Teikyo University, 医学部, リサーチフェロー (10393000)
• Project Period (FY): 2007 – 2008
• Project Status: Completed (Fiscal Year 2008)
• Budget Amount: ¥3,620,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥420,000); Fiscal Year 2008: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000); Fiscal Year 2007: ¥1,800,000 (Direct Cost: ¥1,800,000)
• Keywords: Wilson病 / 肝臓内銅濃度 / アポトーシス / ミトコンドリア損傷 / 肝臓組織学的検索 / LECラット

Research Abstract

Wilson病は肝臓に銅が蓄積することにより様々な障害を引き起こす。本研究では本症の肝障害の発症機構を明らかとし、将来的に肝障害への移行を予防する治療を検討する事が目的である。モデルラットを用いて検討した結果、生後早期から肝臓に銅が蓄積しミトコンドリア障害が誘導されていると考えられた。また、酸化ストレス制御機構が低下している可能性が示唆された。本研究結果から本症ではレドックス制御の正常化による治療効果の検討が必要であると考えられる。

Research Products

• [Journal Article] The first reported case of Menkes disease caused by an Alu insertion mutation. (2007)
  • Author(s): Gu, Y・Kodama, H・Watanabe, S・Kikuchi, N・Ishitsuka, I・Ozawa, H・Fujisawa, I・Ozawa, H・Fujisawa, C・Shiga, K.
  • Journal Title: Brain Dev. 29(2) Pages: 105-8
  • NAID: 10024134962
  • Peer Reviewed
• [Journal Article] Direct Analysis of Ceruloplasmin in Human Blood Serum by HPLC/Inductively Coupled Plasma-Mass Spectrometry for the Diagnosis of Wilson Disease (2007)
  • Author(s): Kobayashi, K・Katsuya, Y・Abdulah, R・Fujisawa, C・Nagamine, T・Morikawa, A・Murakami, M・Kodama, H・Koyama, H
  • Journal Title: Biomed Res Trace Elements 18(1) Pages: 91-95
  • NAID: 10018911132
  • Peer Reviewed
• [Journal Article] Copper Metabolism and Copper Transport Disorders (2007)
  • Author(s): Kodama, H・Fujisawa, C・Gu, YH・Shiga, K
  • Journal Title: Biomed Res Trace Elements 18(3) Pages: 249-254
  • NAID: 10019766046
  • Peer Reviewed
• [Journal Article] Possible associtation of Wilson's disease with hepatocellular carcinoma
  • Author(s): Gu YH, Kodama H, Kato T, Fui isawa C, Kaga F
  • Journal Title: Cell Biology and Toxicology (in press)
  • Peer Reviewed
• [Journal Article] Possible associtation of Wilson's disease with hepatocellular carcinoma
  • Author(s): Gu, YH・Kodama, H・Kato, T・Fujisawa, C・Kaga, F
  • Journal Title: Cell Biology and Toxicology (In press)
  • Peer Reviewed
• [Presentation] 低体重児におけるカルシウム及び骨代謝関連ミネラル・微量元素の必要摂取量に関する研究 (2008)
  • Author(s): 藤澤千恵、井浦綾、加賀文彩、志賀勝秋、坂良逸、児玉浩子
  • Organizer: 第5回日本小児栄養研究会
  • Place of Presentation: 奈良
  • Year and Date: 2008-03-01
• [Presentation] Studies on Mutations in ATP7A Gene in Male Patients and a Female P atient with Menkes Disease (2008)
  • Author(s): Hiroko Kodama, Chie Fujisawa, Yag-Hong Gu, Katsuaki Shiga, F umiava Ka ea
  • Organizer: The 4th Congress of Asian Society for Plediatric Research
  • Place of Presentation: Hawaii
• [Presentation] Copper and zinc status in the breast milk of mothers with Wilson di ase (2008)
  • Author(s): Kaga F, Kodama H, Siga K, Fuj isawa C, Yanakawa Y, Kobayash i K, Koyama H
  • Organizer: Annual Symposium of the Society for the Study o f Inborn Errors of Metabolism
  • Place of Presentation: Lisboa, Portugal
• [Presentation] Higher copper accumulation, a possible cause for hepatocelluar carcinom a in patients with wilson disease (2008)
  • Author(s): Kodama H, Shiga K, Kaga F, F uiisawa C, Gu YH
  • Organizer: Annual Symposium of the Society for the Study c f Inborn Errors of Metabolism
  • Place of Presentation: Lisboa, Portugal
• [Presentation] 低出生体重児の血清カルシウム、微量元素の経時的変化 (2007)
  • Author(s): 藤澤千恵、井浦綾、太田久吉、加賀文彩、志賀勝秋、坂良逸、玉井浩、児玉浩子
  • Organizer: 第18回日本微量元素学会
  • Place of Presentation: 福井
  • Year and Date: 2007-07-05
• [Presentation] メンケス病の遺伝子解析と母親の保因者率 (2007)
  • Author(s): 小泉亮輔、鎌田和、加賀文彩、志賀勝秋、顧艶紅、藤澤千恵、児玉浩子
  • Organizer: 第11回ウィルソン病研究会
  • Place of Presentation: 東京
  • Year and Date: 2007-05-12
• [Presentation] Expectation of a New Therapy for Menkes Disease by Speciation, Metabolomics and Metallomics on Copper (2007)
  • Author(s): Hiroko, Kodama ・Chie, Fujisawa・Yag-Hoong Gu ・Katsuaki, Shiga・Fumiaya, Kaga
  • Organizer: International Symposium on Metallomics 2007
  • Place of Presentation: Nagoya