| Project/Area Number |
06454304
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| Research Category |
Grant-in-Aid for General Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Nagoya City University |
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Principal Investigator |
WADA Yoshiro Nagoya City University, School of Medicine, Professor, 医学部, 教授 (30004849)
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| Co-Investigator(Kenkyū-buntansha) |
AGUI Takashi Nagoya City University, School of Medicine, Associate Professor, 医学部, 助教授 (00212457)
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| Project Period (FY) |
1994 – 1995
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| Project Status |
Completed (Fiscal Year 1995)
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| Budget Amount *help |
¥7,200,000 (Direct Cost: ¥7,200,000)
Fiscal Year 1995: ¥1,900,000 (Direct Cost: ¥1,900,000)
Fiscal Year 1994: ¥5,300,000 (Direct Cost: ¥5,300,000)
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| Keywords | Wilson's desease / Gene therapy / LEC rats |
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| Research Abstract |
Restriction fragment length polymoyphism on the Wilson's disease gene betweem LEC and control WKAH rats was found by using a human Wilson's disease cDNA fragment amplified with polymerase chain reaction as the probe. This RFLP was found to show a cross-linkage with the hepatic disorder in back-crosses between LEC and WKAH rats, indicating that the hepatic disorder in LEC rats is due to the same gene as the case of human Wilson's disease. This result suggests that LEC rats are usuful animal model for the Wilson's disease gene therapy.
In order to construct a retro virus vector inserted the human Wilson's disease gene for the gene therapy, we tried cloning of this gene cDNA from the cDNA library made from the healthy human liver. However, cDNA clones obtained all did not encode the whole coding region, possibly due to the relatively long coding region of this gene. We are under re-screening the cDNA library to obtain cDNA encoding the whole coding region.
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