Detatorubral-pallidoluysian atrophy: relationship between the size of the CAG repeat expansion and the pathological changes
| Project/Area Number |
08680819
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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 |
Nerve anatomy/Neuropathology
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| Research Institution | Tottori University |
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Principal Investigator |
OHAMA Eisaku Tottri University, Neuropathology, Professor, 医学部, 教授 (50018892)
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| Co-Investigator(Kenkyū-buntansha) |
KATO Shinsuke Tottri University, Neuropathology, Associate Professor, 医学部, 講師 (60194817)
SHIMOMURA Tokio Tottri University, Clinical laboratory medicine, Associate professor, 医学部, 助教授 (00216136)
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| Project Period (FY) |
1996 – 1997
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| Project Status |
Completed (Fiscal Year 1997)
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| Budget Amount *help |
¥2,500,000 (Direct Cost: ¥2,500,000)
Fiscal Year 1997: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 1996: ¥1,300,000 (Direct Cost: ¥1,300,000)
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| Keywords | Dntatorubral-pallidoluysian atrophy / Autopsy material / paraffin section / number of CAG repeats / pathological changes |
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| Research Abstract |
We investigated the relationship between the size of the CAG repeat expansion and the pathological changes in 13 autopsy cased of dentatorubral-pallidoluysian atrophy (DRPLA). The size of the CAG repeat was determined using formalin-fixed and paraffin embedded sections of the frontal love according to the method reported by Koide et al and Nagatuchi et al. In all 13 cases the size of the CAG repeat was expanded from 55 to 80. The degree of neuronal loss and glosses was evaluated in the dentate nucleus and globes plaids as well as other affected sites such as putamen, caudate nucleus, cerebral and cerebellar cortices, thalamus, substantia nigra, and spinal cord. Brain weight and the degree of brain stem atrophy were also evaluated. There was no correlation with the size of the CAG repeat and the severity of the pathological changes in 13 autopsy cases of DRPLA examined.
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Report
(3 results)
Research Products
(11 results)
