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1991 Fiscal Year Final Research Report Summary

Studies on polymorphism of the prion protein gene in familial Creutzfeldt-Jakob disease.

Research Project

Project/Area Number 02454245
Research Category

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

Allocation TypeSingle-year Grants
Research Field Neurology
Research InstitutionKyusyu University

Principal Investigator

TATEISHI Jun  Kyushu University, Faculty of Medicine, Professor, 医学部, 教授 (70033305)

Co-Investigator(Kenkyū-buntansha) KITAMOTO Tesuyuki  Kyushu University, Faculty of Medicine, Lecturer, 医学部, 講師 (20192560)
Project Period (FY) 1990 – 1991
KeywordsCreutzfeld-Jakob disease / Gerstmann-Straussler syndrome / prion protein / polymorphism
Research Abstract

We examined polymorphism of prion protein open reading frame. We found out several point mutations and insertional polymorphism. At first, we reported that codon 102 or codon 117 point mutation was linked to Gerstmann-Straussler syndrome. We also found codon 129 mutation, codon 200 mutation and 168 bp insertion in the proline-glycine rich repetitive portion of prion protein. To examine the precise localization of prion protein, we established a new pretreatment. designated as hydrolytic autoclaving. This pretreatment could reveal abnormal prion protein accumulations in the gray matter of the central nervous system. This diffuse gray matter stainings were documented in all patients with Creutzfeldt-Jakob disease. Prion protein immunostainings were similar to synaptophysin immunostainings. To examine precisely, we performed double immunolabelling using anti-prion protein and anti-synaptophysin. Both immunoreactions were colocalized in the same synaptic structures. Therefore, we reported … More that the major localization of abnormal prion protein is the synaptic structures in patients with sporadic Creutzfeldt-Jakob disease.
We immunohistochemically examined tissue sections from patients with prion protein polymorphism using hydrolytic autoclaving enhancement. Abnormal prion protein accumulations could be classified into plaque formations(plaque-type)and the diffuse gray matter stainings including synaptic structures(synaptic-type). Both insertional polymorphism and a point mutation in codon 102, 117/129, 129 result in plaque-type prion protein accumulations. The patients with codon 102 mutation also have synaptic type prion protein accumulations. However, a point mutation in codon 200 did not show plaque-type accumulations, and only showed synaptic-type prion protein accumulations. Likewise, sporadic Creutzfeldt-Jakob disease patients without any known mutations only have synaptic-type accumulations. These results imply that the primary structures of prion protein influence in the phenotype of prion protein disease, especially in abnormal prion protein distributions of the central nervous system. Less

  • Research Products

    (46 results)

All Other

All Publications (46 results)

  • [Publications] Doerr-Schott J: "Immunogold light and electron microscopic detection of amyloid plaques in transmissible spongiform encephalopathies." Neuropathol.appl.Neurobiol.16. 85-89 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto T: "Cerebral amyloid in mice with Creutzfeldt-Jakob disease is influenced by the strain of the infections agent." Brain Res.508. 165-167 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ogomori K: "Brain amyloid in aging and Alzheimer's disease." Review of Biological Research on Aging. 4. 171-179 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Doh-ura K: "Creutzfeldt-Jakob disease patients with Congophilic kuru plaques have the missense variant protein common to Gerstmann-Straussler syndrome." Ann.Neurol.27. 121-126 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Tateishi J: "Immunochemical,Molecular genetic,and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome." Neurology. 40. 1578-1581 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Shin RW: "Modified tau is present in younger nondemented persons:A study of subcortical nuclei in Alzheimer's disease and progressive supranuclear palsy." Acta Neuropathol.81. 517-523 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ohgami T: "Incresed senile without microglia in Alzheimer's disease." Acta Neuropathol.81. 242-247 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Shin RW: "Hydrated autoclave pretreatment enhances tau immunoreactivity in formalin-fixed normal and Alzheimer's disease brain tissues." Lab.Invest.64. 693-702 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto T: "N-terminal sequence of prion protein is also integrated in kuru plaques of patients with Gerstman-Straussler syndrome." Brain Res.545. 319-321 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto T: "A prion protein missense variant is integrated in kuku plaque cores in patients with Gerstmann-Straussler syndrome." Neurology. 41. 306-310 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Tateishi J: "Practical methods for chemical inactivation of Creutzfeldt-Jakob disease pathogen." Mirobiol.Immunol.35. 163-166 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Miyazono M: "A comparative immunohistochemical study of kuru and senile plaques with a special reference to glial reactions at various stages of amyloid plaque formation." Am.J.Pathol.139. 589-598 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Doi-Yi R: "Immunoreactivity of cerebral amyloidosis is enhanced by protein denaturation treatment." Acta Neuropathol.82. 260-265 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Doh-ura K: "CJD discrepancy." Nature. 353. 801-802 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto T: "Abnormal isoform of prion protein accumulates in follicular dendritic cells mice with Creutzfeldt-Jakob disease." J.Virol. 65. 6292-6295 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ohgami T: "Alzheimer's amyloid precursor protein-positive degenerative neurites exist even within kuru plaques not specific to Alzheimer's disease." Am.J.Pathol.139. 1245-1250 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kretzschmar HA: "Diffuse deposition of immunohistochemically labeled prion protein in the granular layer of the cerebellum in a patient with Creutzfeldt-Jakob disease." Acta Neuropathol.82. 536-540 (1991)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Tateishi J: "Creutzfeldt-Jakob disease with amyloid angiopathy;diagnosis by immunological analyses and transmission experiments." Acta Neuropathol.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto T: "Abnormal isoform of prion protein accumulates in the synaptic structures in patients with Creutzfeldt-Jakob disease." Am.J.Pathol.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Miyazono M: "Colocalization of prion protein and protein in the same amyloid plaque in patient with Gerstmann-Straussler syndrome." Acta Neuropathol.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Muramoto T: "The sequencial development of abnormal prion protein acumulations in mice with Creutzfeldt-Jakob disease." Am.J.Pathol.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ohgami T: "Alzheimer's amyloid precursor protein accumulates within axonal swellings in human brain lesions." Neurosci.Lett.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Tateishi J: "Handbook of cerebellar diseases" Marcel Dekker, (1992)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Doerr-Schott J, Kitamoto T, Tateishi J, Boellaard JW, Heldt N, Lichte C: "Immunogold light and electron microscopic detection of amyloid plaques in tranissible spongiform encephalopathies." Neuropathol. appl. Neurobiol.16. 85-89 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto T, Yi R, Mohri S, Tateishi J: "Cerebral amyloid in mice with Creutzfeldt-Jakob disease is influenced by the strain of the infections agent." Brain Res. 508. 165-167 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ogomori K, Kitamoto T, Tateishi J: "Brain amyloid in aging and Alzheimer's disease." Review of Biological Research on Aging. 4. 171-179 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Dohura K, Tateishi J, Kitamoto T, Sasaki H, Sakaki Y: "Creutzfeldt-Jakob disease patients with congophilic kuru plaques have the missense variant protein common to Gerstmann-Straussler syndrome." Ann. Neurol. 27. 121-126 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Tateishi J, Kitamoto T, Dohura K, Sakaki H, Steinmetz G, Tranchant C, Warter JM, Heldt N: "Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Straussler-Scheinker syndrome" Neurology. 40. 1578-1581 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shin RW, Kitamoto T, Tateishi J: "Modified tau is present in younger nondemented persons : A study of subcortical nuclei in Alzheimer's disease and progressive supranuclear palsy." Acta Neuropathol. 81. 517-523 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ohgami T, Kitamoto T, Shin RW, Kaneko Y, Ogomori K, Tateishi J: "Increased senile without microglia in Alzheimer's disease." Acta Neuropathol. 81. 242-247 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shin RW, Iwaki T, Kitamoto T, Tateishi J: "Hydrated autoclave pretreament enhances tau immunoreactivity in formalin-fixed normal and Alzheimer's disease brain tissues." Lab. Invest.64. 693-702 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto T, Muramoto T, Hilbich C, Beyreuther K, Tateishi J: "N-terminal sequence of prion protein is also integrated in kuru plaqus of patients with Gerstmann-Straussler syndrome." Brain Res. 545. 319-321 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto T, Yamaguchi K, Dohura K, Tateishi J: "A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Straussler syndrome." Neurology. 41. 306-310 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Tateishi J, Tashima T, Kitamoto T: "Practical methods for chemical inactivation of Creutzfeldt-Jakob disease pathogen." Microbiol. Immunol.35. 163-166 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Miyazono M, Iwaki T, Kitamoto T, Kaneko Y, Dohura K, Tateishi J: "A comparative immunohistochemical study of kuru and senile plaques with a special reference to glial reactions at various stages of amyloid plaque formation." Am. J. Pathol.139. 589-598 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Doi-Yi R, Kitamoto T, Tateishi J: "Immunoreactivity of cerebral amyloidosis is enhanced by protein denaturation treatment." Acta Neuropathol.82. 260-265 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Dohura K. Kitamoto T, Sakaki Y, Tateishi J: "CJD discrepancy." Nature. 353. 801-802 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto T, Muramoto T, Mohri S, Dohura K, Tateishi J: "Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease." J. Virol. 65. 6292-6295 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ohgami T, Kitamoto T, Weidemann A, Beyreuther K, Tateishi J: "Alzheimer's amyloid precursor protein-positive degenerative neurites exist even within kuru plaques not specific to Alzheimer's diseases." Am. J. Pathol. 139. 1245-1250 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kretzschmar HA, Kitamoto T, Doerr-Schott J, Mehrain P, Tateishi J: "Diffuse deposition of immunohistochemically labeled prion protein in the granular layer of the cerebellum in a patient with Creutzfeldt-Jakob disease." Acta Neuropathol.82. 536-540 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Tateishi J, Kitamoto T, Dohura K, Boellaard JW, Peiffer J: "Creutzfeldt-Jakob disease with amyloid angiopathy ; diabnosis by immunological analyses and transmission experiments." Acta Neuropathol.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto T, Shin RW, Dohura K, Tomokane N, Miyazono M, Muramoto T, Tateishi J: "Abnormal isoform of prion protein accumulates in the synaptic structures in patients with Creutzfeldt-Jakob disease." Am. J. Pathol.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Miyazono M, Kitamoto T, Iwaki T, Tateishi J: "Colocalzation of prion protein and protein in the same amyloid plaque in patient with Gerstmann-Strallssler syndrome." Acta Neuropathol.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Muramoto T, Kitamoto T, Tateishi J, Goto I: "The sequential development of abnormal prion protein accumulations in mice with Creutzfeldt-Jakob disease." Am. J. Pathol.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ohgami T, Kitamoto T, Tateishi J: "Alzheimer's amyloid precursor protein accumulates within axonal swellings in human brain lesions." Neurosci. Lett.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Tateishi J, Kitamoto T, Dohura K: "Slow transmissible diseases affecting the cerebellum." In Handbook of cerebellar diseases, ed. by Lechtenberg R, Marcel Dekker, New York. (1992)

    • Description
      「研究成果報告書概要(欧文)」より

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Published: 1993-03-16  

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