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Detatorubral-pallidoluysian atrophy: relationship between the size of the CAG repeat expansion and the pathological changes

Research Project

Project/Area Number 08680819
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionTottori University

Principal Investigator

OHAMA Eisaku  Tottri University, Neuropathology, Professor, 医学部, 教授 (50018892)

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)
Project Period (FY) 1996 – 1997
Project Status Completed (Fiscal Year 1997)
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)
KeywordsDntatorubral-pallidoluysian atrophy / Autopsy material / paraffin section / number of CAG repeats / pathological changes
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.

Report

(3 results)
  • 1997 Annual Research Report   Final Research Report Summary
  • 1996 Annual Research Report
  • Research Products

    (11 results)

All Other

All Publications (11 results)

  • [Publications] 下村登規夫: "剖検脳材料からの遺伝子診断ーパラフィン切片からの診断"医学のあゆみ. 176・8. 490-491 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 大浜栄作: "ビンスワンガー型痴呆の病理学的特徴"Geriat Med (老年医学). 35・5. 554-560 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Tokio Shimomura, Eisaku Ohama: "Genetic diagnosis from autopsied brain tissue using paraffin sectons."J Clin Exp Med. 176-3. 490-491 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Eisaku Ohama, Shinichiro Tanaka, Yo Horikawa, Takashi Morita: "Pathological characteristics of Binswanger's disease"Geriat Med. 35-5. 554-560 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shinsuke Kato: "Pathological Characterization of astrocytic inclusions in familial amyotrophi lateral sclerosis." Am.J.Pathol.151・2. 611-620 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 大浜栄作: "ビンスワンガー型痴呆の病理学的特徴" Geriat Med. 35・5. 554-560 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Yasuhiro Watanabe: "Absence of mutant SOD1 in familial amyotrophic lateral sclerosis with two base pair deletion in SOD1 gene." Acta Neurol Scand. 95・2. 167-172 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 下村登規夫: "剖検材料からの遺伝子診断-パラフィン切片からの診断" 医学のあゆみ. 176・8. 490-491 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Shinsuke Kato: "Familial amyotrophic lateral sclerosis with a two base pair delesion in superoxide dismutase 1 gene." J.Neuropathol.Exp.Neurol.55.10. 1089-1101 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Masaru Shimoda: "Pallidonigroluysian atrophy:clinicopathological and immunohistochemical studies." Neuropathology. 16.1. 21-28 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Masayuki Ito: "Morphological study of the brainstem in Fukuyama type congenital musuclar distrophy." Pediatr.Neurol. 15.4. 327-331 (1996)

    • Related Report
      1996 Annual Research Report

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Published: 1996-04-01   Modified: 2016-04-21  

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