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Specificity of ubiquitin-positive inclusions in amyotrophic lateral sclerosis

Research Project

Project/Area Number 05670578
Research Category

Grant-in-Aid for General Scientific Research (C)

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionNihon University School of Medicine

Principal Investigator

MIZUTANI Tomohiko  Nihon Univ., Neurology, Associate professor, 医学部・神経学教室, 助教授 (00166018)

Co-Investigator(Kenkyū-buntansha) KAKIMI Shigeo  Nihon Univ., Anatomy, Assistant, 医学部・第2解剖学教室, 助手 (40060165)
Project Period (FY) 1993 – 1994
Project Status Completed (Fiscal Year 1994)
Budget Amount *help
¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1994: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 1993: ¥1,000,000 (Direct Cost: ¥1,000,000)
Keywordsubiquitin / filamentous inclusion / amyotrophic lateral sclerosis / lymphoma / skein-like inclusion / ユビキチン陽性封入体 / 運動ニューロン疾患
Research Abstract

Ubiquitin-positive filamentous inclusions have been considered to represent abnormalities specific to amyotrophic lateral sclerosis (ALS). However, the inclusions have not yet been fully investigated in other neurological diseases and non-neurological controls. To evaluate the specificity of the filamentous inclusions, we studied, both immunohistochemically and immunoelectron microscopically, the motor neurons of the lumbosacral cords from 23 patients with ALS,31 patients with 19 different kinds of other neurological diseases (non-ALS) and 37 controls. We stained paraffin-embedded sections of the spinal cords by the avidin-biotin complex method using polyclonal anti-ubiquitin anti-bodies, and examined the anterior horn motor neurons. In ALS,we found 3 kinds of inclusions, i.e.filamentous and granular inclusions, and dense round bodies corresponding to Lewy bodies. Filamentous inclusions were observed in 19 of 23 patients with ALS,and in 1 of 31 non-ALS patients, but were not found in any of the 37 controls. Filamentous inclusions were more frequently observed in ALS patients than in non-ALS patients or controls (p<0.01). The non-ALS patient with filamentous inclusions had a B cell lymphoma which infiltrated the proximal parts of the peripheral nerves, causing marked central chromatolysis in the anterior horn motor neurons. On immunoelectron microscopy, the granular inclusions in ALS contained not only the constituents similar to those of the filamentous inclusions in ALS,but also lipofuscin granules, whereas the filamentous inclusions in the lymphoma patient showed the involvement of ribosomes. Although our data regarding the frequency of the filamentous inclusions further indicate the specificity of the inclusions in ALS,similar, if not identical, inclusions may also be observed in non-ALS patients. This suggests that ubiquitin-positive filamentous inclusions are characteristic of, but not pathognomonic of ALS.

Report

(3 results)
  • 1994 Annual Research Report   Final Research Report Summary
  • 1993 Annual Research Report
  • Research Products

    (12 results)

All Other

All Publications (12 results)

  • [Publications] Tomohiko MIZUTANI Yutaka YAMAGUCHI Toshiaki TAKASU: "Ubiquitin-positive inclusions in amyotrophic lateral sclerosis and other neurdogical diseases" Neuropathology. 13(Suppl). 91- (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko MIZUTANI Yutaka YAMAGUCHI Toshiaki TAKASU: "Specificity of ubiquitin-positive inclusions in amyotrophic lateral sclerosis" Canadian Journal of Neurological Sciences. 20(Suppl). S107- (1993)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko MIZUTANI Shigeo KAKIMI Yutaka YAMAGUCHI Toshaki TAKASU: "Ubiquitin-positive inclusions in amyotrophic lateral sclerosis and other neurdogical diseases" Brain Pathology. 4. 528- (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水谷智彦: "神経内科モダンコンセプト5" 医学書院 東京, 238 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水谷智彦: "脊椎脊髄疾患" 三輪書店 東京(発行予定), 未定

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko Mizutani, Yutaka Yamaguchi, Toshiaki Takasu: "Ubiquitin-positive inclusions in amyotrophic lateral sclerosis and other neurological diseases" Neuropathology. 13 (Suppl). 91 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko Mizutani, Yutaka Yamaguchi, Toshiaki Takasu: "Specificity of ubiquitin-positive inclusions in amyotrophic lateral sclerosis" Canadian Journal of Neurological Sciences. 20 (Suppl). S107 (1993)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko Mizutani, Shigeo Kakimi, Yutaka Yamaguchi, Toshiaki Takasu: "Ubiquitin-positive inclusions in amyotrophic lateral sclerosis and other neurological diseases" Brain Pathology. 4. 528 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko Mizutani: Pathology of Degenerative Diseases. Modern Concept Neurology 5 ed.by Tahiro K,Mizuno Y,Kurihara T,IGAKUSHOIN,Tokyo, Japan, 197-208 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Tomohiko Mizutani: Degenerative Diseases of the Spinal Cord. Spine and Spinal Cord Diseases ed.by Ito T,Hattori T,Yamaura A MIWA SHOTEN,Tokyo (in press),

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] 水谷智彦: "脊椎脊髄疾患(伊藤達雄、服部孝道、山浦晶編)" 三輪書店、東京(発行予定),

    • Related Report
      1994 Annual Research Report
  • [Publications] 水谷智彦: "モダンコンセプト神経内科5(田代邦雄、水野美邦、栗原照幸編)" 医学書院、東京(発行予定),

    • Related Report
      1994 Annual Research Report

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Published: 1993-04-01   Modified: 2016-04-21  

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