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The study on a new therapy of Menkes disease

Research Project

Project/Area Number 10670759
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionTeikyo University

Principal Investigator

KODAMA Hiroko  Teikyo University, School of Medicine, Department of Pediatrics, Associate Professor, 医学部, 助教授 (00093386)

Co-Investigator(Kenkyū-buntansha) TAKAHASHI Ichiro  Teikyo University, School of Medicine, Department of Pediatrics, Assistant Professor, 医学部, 講師 (40091045)
TAMURA Hiroshi  Teikyo University, School of Medicine, Department of Pediatrics, Associate Professor, 医学部, 助教授 (30101728)
Project Period (FY) 1998 – 1999
Project Status Completed (Fiscal Year 1999)
Budget Amount *help
¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 1999: ¥1,300,000 (Direct Cost: ¥1,300,000)
Fiscal Year 1998: ¥1,800,000 (Direct Cost: ¥1,800,000)
KeywordsMenkes disease / copper / chelating agent / threatment / macular mouse / macular マウス / 銅輸送ATPase / 銅投与 / シチオカーボ
Research Abstract

Menkes disease is a neurodegenerative disorder characterized by a copper deficiency in various organs including the brain and liver. On the contrary, copper accumulated in the kidney and intestine. These abnormalities of copper distribution are caused by a reduced membrane-transport of copper. We examined effects of a combination therapy of a subcutaneous copper administration and an oral administration of a chelating agent (N,N-diethyl carbamirate) on copper metabolism in the macular mouse, an animal model of Menkes disease. 【Materials and Methods】 Four weeks old macular mice and control mice were used. Copper (20μg/time) and diethyl carbamirate (0.05 mg/g body weight/time) were given twice a week for 4 weeks. After that, the mice were sacrificed, and the brain, liver, kidney and intestine were obtained. Copper concentrations in those tissues were analyzed with an atomic absorption spectrophotometer. The activity of cytochrome C oxidase in those tissues was also examined. 【Results and Discussion】 The copper concentration were improved in the brain and liver of macular mice that were treated with a combination of copper and a chelating agent. The copper concentrations were not changed in the kidney and intestine of the treated macular mice. However, the activities of cytochrome C oxidase were decreased in the brain and kidney of macular mice that were treated with the combination therapy. These results suggest that copper distribution was improved with the combination therapy, however, the copper enzyme activities were not improved by the therapy.

Report

(3 results)
  • 1999 Annual Research Report   Final Research Report Summary
  • 1998 Annual Research Report
  • Research Products

    (29 results)

All Other

All Publications (29 results)

  • [Publications] Murata Y, Kodama H, et al.: "Mottled gene expression and copper distribution in the mucular mouse, an animal model of Menkes disease"J Inhert Metab Dis. 21. 199-202 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, et al.: "Molecular genetics and pathophysiology of Menkes disease"Pediatr Internal. 41. 430-435 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, et al.: "Clinical manifestations and treatment of Menkes disease and its variants"J Inhert Metab Dis. 41. 423-429 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 児玉浩子: "ヒトのメンケス病、後角症候群とMottledミュータント"脳の科学. 21. 177-181 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 鵜沢祐貴子、児玉浩子ら: "Menkes病でのATP7A活性と遺伝子異常"Biomed Res Trace Elements. 10. 201-202 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 代田道彦、児玉浩子ら: "Menkes病における骨代謝マーカーの検討"Biomed Res Trace Elements. 10. 155-156 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 児玉浩子(分担): "小児科の進歩19"診断と治療社. 47-50 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 児玉浩子(分担): "遺伝治療開発ハンドブック"日本遺伝子治療学会. 1008-1012 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Murata Y, Kodama H, Kobayashi M, Abe T.: "Mottled gene expression and copper distribution in the macular mouse, an animal model for Menkes disease"J Inher Metab Dis.. 21. 199-202 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, Murata Y.: "Molecular genetics and pathophysiology of Menkes disease"Pediatr International. 41. 430-435 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, Murata Y, Kobayashi M,: "Clinical manifestations and treatment of Menkes disease and its variants"Pediatr International. 41. 423-429 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, Murata Y, Kobayashi M, Mochizuki D, Abe T.: "Copper metabolism and mutation of patients with Menkes disease."5th conference of the International Society for Trace Element Research in Hunams. Lyon. (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Murata Y, Kodama H, Kobayashi M, Mochizuki D, Abe T.: "Mottled gene expression in normal Ed macular mouse, Model of Menkes disease."5th conference of the International Society for Trace Element Research in Hunams. Lyon. (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Kodama H, Murata Y, Mochizuki D, Kobayashi M, Yanagawa Y.: "Copper Metabolism and Mutation Analysis in Patients with Menkes Disease and Occipital Horn Syndrome."2nd Copper Homeostasis and Its Disorders: Molecular and Cellular Aspects. Ravello, Italy. 17-21 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Shirota M, Kodama H, Kobayashi M, Mochizuki D, Yanagawa Y.: "Connective Tissue Abnormalities in Patients with Menkes Disease"Society for the Study of Inborn Errors of Metabolism 37th Annual Symposium. Genova. 7-10 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Murata Y,Kodama H,et al.: "Mottled gene expression and copper distribution in the mucular mouse, an animal model of Menkes disease"J inhert Metab Dis. 21. 199-202 (1998)

    • Related Report
      1999 Annual Research Report
  • [Publications] Kodama H,et al.: "Molecular genetics and pathophysiology of Menkes disease"Pediatr Internal. 41. 430-435 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Kodama H. et al.: "Clinical manifestations and treatment of Menkes disease and its variants"Pediatr Internal. 41. 423-429 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 児玉浩子: "ヒトのメンケス病、後角症候群とMottledミュータント"脳の科学. 21. 177-181 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 鵜沢祐貴子、児玉浩子ら: "Menkes病でのATP7A活性と遺伝子異常"Biomed Res Trace Elements. 10. 201-202 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 代田道彦、児玉浩子ら: "Menkes病における骨代謝マーカーの検討"Biomed Res Trace Elements. 10. 155-156 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 児玉浩子(分担): "小児科の進歩 19"診断と治療社. 4 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 児玉浩子(分担): "遺伝子治療開発ハンドブック"日本遺伝子治療学会. 5 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 村田佳子,西脇れい,児玉浩子ら: "Menkes病患者の遺伝子解析と銅代謝" 日本先天代謝異常学会雑誌. 14(2). 260 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] 村田佳子,児玉浩子: "Menkes病の遺伝子解析と病態との関連" 日本小児科学会雑誌. 103(2). 147 (1999)

    • Related Report
      1998 Annual Research Report
  • [Publications] H.KODAMA et.al: "Structure and function of ATP7A as studied by mutotion analysis" 生体機能における金属イオンの特異的作用の分子科学. 3. 48 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] 児玉浩子: "ヒトのMenkes病 後角症候群とマクラ・マウス" 脳の科学. 増刊. (1999)

    • Related Report
      1998 Annual Research Report
  • [Publications] 児玉浩子(分担): "小児科学年鑑 1999" 診断と治療社, (1999)

    • Related Report
      1998 Annual Research Report
  • [Publications] 児玉浩子(分担): "遺伝子治療開発研究ハンドブック" 日本遺伝子治療学会(予定), (1999)

    • Related Report
      1998 Annual Research Report

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Published: 1998-04-01   Modified: 2016-04-21  

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