2003 Fiscal Year Final Research Report Summary
Introduction of human mini-chromosome vector carrying human dystrophin gene into the mdx mice
| Project/Area Number |
14580791
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
神経・脳内生理学
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| Research Institution | Kitasato University |
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Principal Investigator |
HANAOKA Kazunori Kitasato Univ., School Sci., Prof., 理学部, 教授 (40189577)
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| Co-Investigator(Kenkyū-buntansha) |
UCHIYAMA Kouji Kitasato Univ., School Sci., 理学部, 助手 (20276174)
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| Project Period (FY) |
2002 – 2003
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| Keywords | HAC / chimera / ES cell / DMD / chromosamal manipulation / mdx mouse / muscular dystrophy |
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| 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.
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Research Products
(6 results)
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[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
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[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
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