| Project/Area Number |
05670575
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | Teikyo University |
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Principal Investigator |
USUKI Fusako Department of Neurology, Teikyo University School of Medicine, Lecturer, 医学部, 講師 (50185013)
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| Co-Investigator(Kenkyū-buntansha) |
SHIMIZU Teruo Department of Neurology, Teikyo University School of Medicine, Professor, 医学部, 教授 (00107666)
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| Project Period (FY) |
1993 – 1994
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| Project Status |
Completed (Fiscal Year 1994)
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| Budget Amount *help |
¥2,100,000 (Direct Cost: ¥2,100,000)
Fiscal Year 1994: ¥800,000 (Direct Cost: ¥800,000)
Fiscal Year 1993: ¥1,300,000 (Direct Cost: ¥1,300,000)
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| Keywords | Glycogenosis type II / Acid alpha-glucosidase (GAA) / Myoblast / Transfer / Therapy / acidalpha-glucosidase(GAA) / GAA前駆体 |
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| Research Abstract |
Acid alpha-glucosidase (GAA) of quails is a 105kD protein and is defective in Japanese quails with glycogenosis type II.GAA is synthesized from a high molecular 130kD precursor protein. GAA precursor is once secreted outside the cell, recaptured by the cell and processed to mature type inside the cell.
We focused on this specific GAA synthesis pathway in order to investigate the possibility of the therapy of glycogenosis type II.In this study we examined whether co-culture of disseased cells with normal myoblasts increased GAA activity in the diseased cells. The formation of hybrid myotubes (90% diseased cells + 10% normal cells) increased GAA activity from 27% of the normal level to 54%. GAA precursor from normal cells seems to be processed in the diseased cells. The reduction of the volume of normal cells remains tobe determined.
We found that GAA activity can be found in the serum in various species. These results suggest that glycogenosis type II in Japanese quails can be easily diagnosed by the direct measurement of the serum GAA activity. The plasmapheresis may be useful for the therapy of glycogenosis type II.
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