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1990 Fiscal Year Final Research Report Summary

Establishment of Prion Protein Immunoassay for Creutzfeldt-Jakob Disease.

Research Project

Project/Area Number 01570198
Research Category

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

Allocation TypeSingle-year Grants
Research Field Experimental pathology
Research InstitutionKyushu University

Principal Investigator

KITAMOTO Tetsuyuki  Kyushu University, Faculty of Medicine, Lecturer, 医学部, 講師 (20192560)

Co-Investigator(Kenkyū-buntansha) TATEISHI Jun  Kyushu University, Faculty of Medicine, Professor, 医学部, 教授 (70033305)
Project Period (FY) 1989 – 1990
KeywordsCreutzfeldt-Jakob disease / prion protein / quantitative analysis / amino acid sequence / amyloid / kuru plaque
Research Abstract

At first, we attempt to clarify the organ distribution of human and murine protease-resistant prion protein in Creutzfeldt-Jakob Disease (CJD), and to measure the concentration of abnormal Prion Protein (PrP) using semi-quantitative Western blot analysis. Human PrP was restricted to the central nervous system, whereas murine PrP was present in the central nervous system and in the lymphoreticular system at the end stage of CJD. PrP concentration in the central nervous system was almost identical to that of humans. The minimum wet weight of an organ with a positive reaction was 0.3 mg for brain, 1 to 3 mg for spleen, 3 mg for spinal cord, 3 mg for lymph node, 10 mg for thymus and 10 to 30 mg for intestine of the CJD-infected mice. There were no immunoreactions in purified PrP fractions form 300 mg of liver, lung or kidney of CJD-infected mice, nor from 300 mg of spleen, lymph node, liver or peripheral nervous system of humans. Within the limits of our method, the distribution of murine … More PrP differed from that of human PrP. Although the immunological detection of PrP does have limits of sensitivity, PrP concentration did correlate with infectivity titers in scrapie infected or CJD-infected mice.
Second, we examined Fukuoka-1 and Fukuoka-2 mouse-adapted CJD strains. Mice infected with Fukuoka-2 strain have a higher incidence of kuru plaques, a higher concentration of PrP, and a higher infectivity titer than do mice with the Fukuoka-1 strain. Thus, it must be kept in mind that there is a difference in the strain of the infectious agent in murine CJD.
Third, to demonstrate that PrP is a component of kuru plaque cores, we fractionated and sequenced kuru plaques core derived peptides, following digestion with Achromobacter lyticus protease I. We identified 3 PrP-derived peptides by reverse-phase high performance liquid chromatography and found a fragment of digests derived from a missense variant of PrP (102 Leu). Variant PrP was also present in the prion rod fraction in patients with Gerstmann-Straussler syndrome. From the data of amino acid sequence, we could not identify the N-terminal sequence because of heterogeneous N-termini. To elucidate whether N-terminal sequence of PrP is related to amyloid formation in vivo, we prepared synthetic N-terminal peptide. Anti-N-terminal antibody immunolabeled kuru plaques positively. Less

  • Research Products

    (32 results)

All Other

All Publications (32 results)

  • [Publications] Ogomori,K.: "Beta protein amyloid is widely distributed in the central nervous system of patients with Alzheimer´s disease." Am.J.Pathol.134. 243-251 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto,T.: "Positive transmission of CreutzfeldtーJakob disease varified by murine kuru plaques." Lab.Invest.60. 507-512 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Shin.R.ーW.: "Increased tau accumulation in senile plaques as a hallmaek in Alzheimer´s disease." Am.J.Pathol.134. 1365-1371 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kaneko,Y.: "Ferritin immunohistochemistry as a marker of microglia." Acta Neuropathol.79. 129-136 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto,T.: "Organ distribution of proteinaseーresisitant prion protein in humans and mice with CreutzfeldtーJakob disease." J.gen Virol.70. 3371-3379 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Dohーura,K.: "Pro→Leu change at position 102 of prion protein is the most common but not the sole mutation related to GerstmannーStraussler syndrome." BioChem.BioPhys.Res.Commun.163. 974-979 (1989)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Doerrー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ー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 Alzheimer´s disease." Rev.Biol.Res.Aging. 4. 171-179 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Dohーura,K.: "CreutzfeldtーJakob disease patients with Congophilic kuru plaques have the missense variant prion protein common to Gerstmann ーStra^^"ussler 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ーStra^^"usslerーScheinker syndrome." Neurology. 40. 1578-1581 (1990)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Kitamoto,T.: "A prison protein missence varient is integrated in kuru plaque cores in patients with GerstmannーStra^^"ussler syndrome." Neurology.

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

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ohgami,T.: "Increased senile plaques without microglia in Alzheimer's disease." Acta Neuropathol.

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Shin,R.ーW.: "Hydrated autoclave pretreatment enhances Tau immunoreactivity in formalinーfixed normal and Alzheimer's disease brain tissue." Lab.Invest.

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

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Ogomori, K.: "Protein amyloid is widely distributed in the central nervous system of patients with Alzheimer's disease." Am. J. Pathol.134. 243-251 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto, T.: "Positive transmission of Creutzfeldt-Jakob disease verified by murine kuru plaques." Lab. Invest. 507-512 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shin, R. -W.: "Increased tau accumulation in senile plaques as a hallmark in Alzheimer's disease." Am. J. Pathol.134. 1365-1371 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kaneko, Y.: "Ferritin immunohistochemistry as a marker for microglia." Acta Neuropathol.79. 129-136 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Kitamoto, T.: "Organ distribution of proteinase-resistant prion protein in humans and mice with Creutzfeldt-Jakob disease." J. Gen. Virol.70. 3371-3379 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Doh-ura, K.: "Pro-> Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler syndrome" Biophys. Res. Common.163. 974-979 (1989)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Doerr-Schotte, 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(欧文)」より
  • [Publications] Kitamoto, T.: "Cerebral amyloid in mice with infectious agent." Brain Res.508. 165-167 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ogomori, K.: "Brain amyloid in aging and Alzheimer's disease." Rev. Biol. Res. on Aging. 4. 171-179 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Doh-ura, K: "Creutzfeldt-Jakob disease patients with Congophilic kuru plaques have the missense variant prion 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.: "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] Kitamoto, T.: "A prion protein missense variant is integrate in kuru plaque cores in patients with Gerstmann-Straussler syndrome." Neurology.

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

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Ohgami, T.: "Modified tau is present in younger nondemented persons ; A study of subcortical nuclei in Alzheimer's disease and" Acta Neuropathol.

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Shin, R. -W.: "Hydrated autoclave pretreatment Tau immunoreactivity in formalin-fixed normal and Alzheimer's disease brain tissues." Lab. Invest.

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

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

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Published: 1993-08-12  

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