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2003 Fiscal Year Final Research Report Summary

Introduction of human mini-chromosome vector carrying human dystrophin gene into the mdx mice

Research Project

Project/Area Number 14580791
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field 神経・脳内生理学
Research InstitutionKitasato University

Principal Investigator

HANAOKA Kazunori  Kitasato Univ., School Sci., Prof., 理学部, 教授 (40189577)

Co-Investigator(Kenkyū-buntansha) UCHIYAMA Kouji  Kitasato Univ., School Sci., 理学部, 助手 (20276174)
Project Period (FY) 2002 – 2003
KeywordsHAC / chimera / ES cell / DMD / chromosamal manipulation / mdx mouse / muscular dystrophy
Research Abstract

Duchenne muscular dystrophy (DMD) is a severe X-linked recessive, progressive muscle-wasting disease caused by mutations in the DMD gene that encodes a 427-kDa cytoskeletal protein called dystrophin. The dystrophin gene is uniquely complex ; 79 exons spread, over approximately 2.5Mb of genomic region, with at least seven separate promoters. Multiple isoforms of dystrophin, thus formed, are expressed in muscle and non-muscle tissues at separate stages of development.
In the present experiment, we have attempted to introduce whole dystrophin genomic region of the human X chromosome into the mdx mice, which is X chromosome-linked recessive myopathic mutants used as human models of DMD. At first, we have isolate embryonic stem cells from the mdx mice and established a new ES cell line, named mdx-1. The mdx-1 ES cells had the ability of forming viable germ line chimeras. Next, whole dystrophin genomic region of the human X chromosome was allowed to translocate, utilizing Cre-loxP system, into a stable human minichromosome vector, HAC-SC20. The dystrophin-cairying minichromosome was then transferred into the mdx-1 ES cells by microcell-mediated chromosome transfer technique. By injecting the ES cells into the mdx blastocysts, we have produced chimeric mice, in which human dystrophin gene might be expressing exclusively.

  • Research Products

    (6 results)

All Other

All Publications (6 results)

  • [Publications] Kazuki Y, Kimura M, Nishigaki R, Kai Y, Okita C, Siroyoshi Y, Schultz T, Tmizuka K, Hanaoka K, Inoue T, Oshimura M: "Human chromosome 21q22.2-qter carries a gene(s) responsible for downregulation of mlc2a and PEBP in Down syndrome model mice"Biochemical and Biophysical Research Communications. 317. 491-499 (2004)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] M Imai, F Hirano, T Sakamoto, K Sekine, Y Mizukura, M Saito, T Kitamoto, M Hayasaka, K Hanaoka, Y Nakagawa: "Early embryonic lethality caused by targeted disruption of the mouse PHGPx gene"Biochem.Biophys.Res.Communi.. 305. 278-286 (2003)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Yamamoto K, Yoshida K, Miyagoe Y, Ishikawa A, Hanaoka K, Nomoto S, Kaneko K, Ikeda S, Takeda S: "Quantitative evaluation of expression of iron-metabolism genes in ceruloplasmin-deficient mice"Biochimica et Biophysica Acta. 1588. 195-202 (2002)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kazuki Y, Kimura M, Nishigaki R, Kai Y, Okita C, Siroyoshi Y, Schultz T, Tomizuka K, Hanaoka K, Inoue T, Oshimura M: "Human chromosome 21q22.2-qter carries a gene(s) responsible for downregulation of mlc2a and PEBP in Down syndrome model mice"Biochem.Biophys.Res.Communi.. 317. 491-499 (2004)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] H Imai, F Hirao, T Sakamoto, K Sekine, Y Mizukura, M Saito, T Kitamoto, M Hayasaka, Ki Hanaoka, Y Nakagawa: "Early embryonic lethality caused by targeted disruption of the mouse PHGPx gene"Biochem.Biophys.Res.Communi.. 305. 278-286 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Yamamoto K, Yoshida K, Miyagoe Y, Ishikawa A, Hanaoka K, Nomoto S, Kaneko K, Ikeda S, Takeda S: "Quantitative evaluation of expression of iron-metabolism genes in ceruloplasmin-deficient mice"Biochimica et Biophysica Acta. 1588. 195-202 (2002)

    • Description
      「研究成果報告書概要(欧文)」より

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Published: 2005-04-19  

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