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PAHTOGENESIS ON MITOCHONDRIAL ANGIOPATHY AND ITS REPAIR BY GENE INJECTION

Research Project

Project/Area Number 12670598
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionFUKUI MEDICAL UNIVERSITY

Principal Investigator

YONEDA Makoto  FUKUI MED. UNIV., University Hospital, LECTURER, 医学部附属病院, 講師 (70270551)

Co-Investigator(Kenkyū-buntansha) KURIYAMA Masaru  FUKUI MED. UNIV., Faculty of Medicine, PROFESSOR, 医学部, 教授 (80107870)
Project Period (FY) 2000 – 2002
Project Status Completed (Fiscal Year 2002)
Budget Amount *help
¥2,800,000 (Direct Cost: ¥2,800,000)
Fiscal Year 2002: ¥600,000 (Direct Cost: ¥600,000)
Fiscal Year 2001: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 2000: ¥1,400,000 (Direct Cost: ¥1,400,000)
KeywordsMELAS / ANGIOPATHY / CYBRIDS / ミトコンドリア血管症 / ミトコンドリア遺伝子変異 / MELAS症候群 / ヒト脳血管内皮細胞
Research Abstract

Mitochondrial encephalplathy, myopathy, lactic acidosis and strokelike episodes (MELAS) syndrome is characterized by a mitochondrial DNA (mtDNA) mutation (A3243G) and mitochondrial angiopathy in the brain. To investigate the pathogenic mechanism for mitochondrial angiopathy, we tried to construct cybrids that carry the MELAS mutation and were derived from human brain vessel endothelial cells. First, we determined the optimal concentration of ethidium bromide (EtBr) to reduce or remove mtDNA from the blood vessel endothelial cells. We then introduced the platelet mitochondria from MELAS patients into the blood vessel endothelial cells lacking mtDNA. Cybrids carrying the mtDNA mutation were hard to survive. We should further investigate the optimal media condition to get cybrids survived.

Report

(4 results)
  • 2002 Annual Research Report   Final Research Report Summary
  • 2001 Annual Research Report
  • 2000 Annual Research Report
  • Research Products

    (18 results)

All Other

All Publications (18 results)

  • [Publications] Tanaka, M et al.: "Gene Therapy for Mitochondrial Disease by Delivering Restriction Endonuclease SmaI into Mitochondria"J Biomed Sci. 9. 534-541 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Yoneda, M et al.: "Late infantile Hirschsprung disease-mental retardation syndrome with a 3-bp deletion in ZFHX1B"Neurology. 59. 1637-1640 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Sahashi, K et al.: "Functional characterization of mitochondrial tRNA-Tyr mutation (5877G-A) associated with familial chronic progressive ophthalmoplegia"J. Med. Genet. 38. 703-705 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Yoshida, K: "Mitochondrial genotypes and radiation-induced micronucleus formation in human osteosarcoma cell in vitro"Oncology Report. 53. 615-619 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Toyoda, E: "Immunosuppresant deoxyspergualin affectts mitochondrial respiratory function in growing cells"J. Antibiotics. 53. 633-636 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Tanaka, M, Borgeld, HJ, Zhang, J, Muramatsu, S, Gong, JS, Yoneda M, Maruyama, W, Naoi, M, Ibi, T, Sahashi, K, Shamoto, M, Fuku, N, Kurata, M, Yamada, Y, Nishizawa, K, Akao, Y, Ohishi, N, Miyabayashi, S, Umemoto, H, Muramatsu, T, Furukawa, K, Kikuchi, A, Nakano, I, Ozawa, K, Yagi, K: "Gene Therapy for Mitochondrial Disease by Delivering Restriction Endonuclease Smal into Mitochondria"J Biomed Sci. 9. 534-541 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Yoneda, M, Fujita, T, Yamada, Y, Yamada, K, Fujii, A, Inagaki, T, Nakagawa, H, Shimada, A, Kishikawa, M, Nagaya, M, Azuma, T, Kuriyama, M, Wakamatsu, N: "Late infantile Hirschsprung disease-mental retardation syndrome with a 3-bp deletion in ZFHX1B"Neurology. 59. 1637-1640 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Sahashi, K, Yoneda, M, Ohno, K, Tanaka, M, Sahashi, K: "Functional characterization of mitochondrial tRNA-Tyr mutation (5877G - A) associated with familial chronic progressive ophthalmoplegia"J Med Genet. 38. 703-705 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Yoshida, K, Yamazaki, H, Ozeki, T, Inoue, T, Yoshioka, Y, Yoneda, M, Fujiwara, Y, Inoue, T: "Mitochondrial genotypes and radiation-induced micronucleus formation in human osteosarcoma cell in vitro"Oncology Report. 53. 615-619 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Toyoda, E, Hiratsuka, M, Nemoto, K, Yoneda, M, Odaka, C: "Immunosuppresant deoxyspergualin affects mitochondrial respiratory function in growing cells"J. Antibiotics. 53. 633-636 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2002 Final Research Report Summary
  • [Publications] Tanaka, M et al.: "Gene Therapy for Mitochondrial Disease by Delivering Restriction Endonuclease SmaI into Mitochondria"J Biomed Sci. 9. 534-541 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Yoneda, M et al.: "Late infantile Hirschsprung disease-mental retardation syndrome with a 3-bp deletion in ZFHX1B"Neurology. 59. 1637-1640 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Yoshida, K, Yamazaki, H, Ozeki, T, Inoue, T, Yoshioka, Y, Yoneda, M.et al.: "Mitochondrial genotypes and radiation-induced micronucleus formation in human osteosarcoma cell in vitro"Oncology Report. 53. 615-619 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Sahashi, K, Yoneda, M, Ohno, K, Tanaka, M, Sahashi, K: "Functional characterization of mitochondrial tRNA-Tyr muration(5877G-A)assocaited with familial chronic progressive ophthalmoplegia"J Med Genet. 38. 703-705 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] 米田誠: "「新ミトコンドリア学」内海耕造,井上正康(編)"共立出版.東京. 10 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Toyoda,E: "Immunosuppresant deoxyspergualin affectts mitochondrial respiratory function in growing cells."J.Antibiotics. 53. 633-636 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 米田誠: "ミトコンドリア異常とてんかん-MERRFを中心に."医学のあゆみ. 193(6). 570-574 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] 藤井明弘: "MELAS患者の脳卒中発作時のADC map."神経内科. 53(9). 436-437 (2001)

    • Related Report
      2000 Annual Research Report

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Published: 2000-04-01   Modified: 2016-04-21  

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