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Cellular Distribution of Prion Protein

Research Project

Project/Area Number 10480211
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionTOHOKU UNIVERSITY

Principal Investigator

KITAMOTO Tetsuyuki  school of medicine, Tohoku University, professor, 大学院・医学系研究科, 教授 (20192560)

Project Period (FY) 1998 – 1999
Project Status Completed (Fiscal Year 1999)
Budget Amount *help
¥13,100,000 (Direct Cost: ¥13,100,000)
Fiscal Year 1999: ¥4,300,000 (Direct Cost: ¥4,300,000)
Fiscal Year 1998: ¥8,800,000 (Direct Cost: ¥8,800,000)
Keywordsprion protein / cellular distribution / conversion mechanism / transgenic mice / embryology / organ-specific factor
Research Abstract

To study the cellular distribution and the mechanism of abnormal conversion of prion protein, we established the transgenic mice with human/mouse chimeric prion protein. Normal cellular form of prion protein in the transgenic mice was easily detected by the immunohistochemical techniques because of high expression level of recombinant prion protein. In the central nervous system, recombinant prion protein was recognized in the axon terminal portion of the neurons. In general organs, we detected recombinant prion protein in the adrenal medulla, the Auerbach's plexus, C cells of the thyroid gland, the anterior pituitary gland, the peripheral nerves, and the myocytes in the heart and the skeletal muscles. We confirmed these recombinant prion protein positive cells were rally prion protein-positive cells in the immunohistochemical analysis with the wild-type mice. In the study of the embryonic state, the cells originated from the neural crest were strongly positive in the prion protein immunolabellings. Among recombinant prion protein-positive cells, we detected the different glycosylation-pattern of prion protein in the Western blot analysis. In the transmission experiment, our animal model was recognized to be a highly sensitive model for human prions. However, it is not possible to detect abnormal isoform of prion protein in general organs except for the central nervous system. Thus, an organ-specific factor to convert the normal cellular form into the abnormal form of prion protein should be clarified in near future.

Report

(3 results)
  • 1999 Annual Research Report   Final Research Report Summary
  • 1998 Annual Research Report
  • Research Products

    (26 results)

All Other

All Publications (26 results)

  • [Publications] Matsuda H.,Mitsuda H.,Nakamura N.,Furusawa S.,Mohri S.,Kitamoto T.: "A chicken monoclonal antibody with specificity for the N-terminal of human prion protein"FEMS Immunol. Med. Microbiol. 23. 189-194 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Shimizu S.,Hoshi K.,Muramoto T.,Homma M.,Ironside JW.,Kuzuhara S.,Sato T.,Yamamoto T.,Kitamoto T.: "Creutzfeldt-Jakob disease with florid plaques after cadaveric dural grafting"Arch. Neurol.. 56. 357-362 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Yamada H.,Itoh Y.,Inaba A.,Wada Y.,Takashima M.,Satoh S.,Kamata T.,Okeda R.,Kayano T.,Suematsu N.,Kitamoto T.,Otomo E.,Matsushita M.,Mizusawa H.: "An inherited prion disease with PrP P105L mutation : clinicopathological and PrP heterogeneity"Neurology. 53. 181-188 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Hainfellner JA.,Parchi P.,Kitamoto T.,Jarius C.,Gambetti P.,Budka H.: "A novel phenotype in familial Creutzfeldt-Jakob disease : Prion protein gene E200K mutation coupled with Valine at codon 129 and type 2 protease-resistant prion protein"Ann. Neurol. 45. 812-816 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Nagashima T.,Okawa M.,Kitamoto T.,Takahashi H.,Ishihara Y.,Ozaki Y.,Nagashima K.: "Wernicke encephalopathy-like symptoms as an early manifestation of Creutzfeldt-Jakob disease in a chronic alcoholic"J. Neurol. Sci. 163. 192-198 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Murayama J.,Shin R-W.,Higuchi J.,Shibuya S.,Muramoto T.,Kitamoto T.: "Interaction of Aluminum with PHFt in Alzheimer's disease neurofibrillary degeneration evidenced by desferrioxamine-assisted chelating autoclave method"Am. J. Pathol. 155. 877-885 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Yamasaki M.,Oyanagi K.,Mori O.,Ohyama M.,Terashi A.,Kitamoto T.,Katayama Y.: "Variant Gerstmann-Straussler syndrome with the P105L prion protein gene mutation : an unusual case with nigral degeneration and widespread neurofibrillary tangles"Acta Neuropathol.. 98. 506-511 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Shibuya S, Shin R-W, Higuchi J, Tateishi J. Kitamoto T: "Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease"Ann Neurol. 43. 826-828 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Shibuya S, Higuchi J, Shin R-W, Tateishi J, Kitamoto T.: "Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease"Lancet. 351. 419 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Matsuda H, Mitsuda H, Nakamura N, Furusawa S, Mohri S, Kitamoto T: "A chicken monoclonal antibody with specificity for the N-terminal of human prion protein. FEMS Immunol."Med. Microbiol.. 23. 189-194 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Shimizu S, Hoshi K, Muramoto T, Homma M, Ironside JW, Kuzuhara S, Sato T, Yamamoto T, Kitamoto T: "Creutzfeldt-Jakob disease with florid plaques after cadaveric dural grafting"Arch. Neurol.. 56. 357-362 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Yamada H. Itoh Y, Inaba A, Wada Y, Takashima M, Satoh S, Kamata T, Okeda R, Kayano T, Suematsu N, Kitamoto T, Otomo E, Matsushita M, Mizusawa H: "An inherited prion disease with PrP P105L mutation : clinicopathological and PrP heterogeneity"Neurology. 53. 181-188 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Hainfellner JA, Parchi P, Kitamoto T, Jarius C, Gambetti P, Budka H.: "A novel phenotype in familial Creutzfeldt-Jakob disease : Prion protein gene E200K mutation coupled with Valine at codon 129 and type 2 protease-resistant prion protein"Ann. Neurol.. 45. 812-816 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Nagashima T, Okawa M, Kitamoto T, Takahashi H, Ishihara Y, Ozaki Y, Nagashima K: "Wernicke encephalopathy-like symptoms as an early manifestation of Creutzfeldt-Jakob disease in a chronic alcoholic"J. Neurol. Sci.. 163. 192-198 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Murayama J, Shin R-W, Higuchi J, Shibuya S, Muramoto T, Kitamoto T: "Interaction of Aluminum with PHFt in Alzheimer's disease neurofibrillary degeneration evidenced by desferrioxamine-assisted chelating autoclave method"Am. J. Pathol.. 155. 877-885 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Yamasaki M, Oyanagi K, Mori O, Ohyama M, Terashi A, Kitamoto T, Katayama Y.: "Variant Gerstmann-Straussler syndrome with the P105L prion protein gene mutation : an unusual case with nigral degeneration and widespread neurofibrillary tangles"

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Matsuda H.,Mitsuda H.,Nakamura N.,Furusawa S.,Mohri S.,Kitamoto T.: "A chicken monoclonal antibody with specificity for the N-terminal of human prion protein"FEMS Immunol. Med. Microbiol. 23. 189-194 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Shimizu S.,Hoshi K.,Muramoto T.,Homma M.,Ironside JW.,Kuzuhara S.,Sato T.,Yamamoto T.,Kitamoto T.: "Creutzfeldt-Jakob disease with florid plaques after cadaveric dural grafting"Arch. Neural. 56. 357-362 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Yamada H.,Itoh Y.,Inaba A.,Wada Y.,Takashima M.,Satoh S.,kamata T.,Okeda R.,Kayano T.,Suematsu N.,Kitamoto T.,Otomo E.,Matsushita M.,Mizusawa H.: "An inherited prion disease with PrP P105L mutation: clinicopathological and PrP heterogeneity"Neurology. 53. 181-188 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Hainfellner JA,Parchi P.,Kitamoto T.,Jarius C.,Gambetti P.,Budka H.: "A novel phenotype in familial Creutzfeldt-Jakob disease: Prion protein gene E200K mutation coupled with Valine at codon 129 and type 2 protease-resistant prion protein"Ann. Neurol. 45. 812-816 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Nagashima T.,Okawa M.,Kitamoto T.,Takahashi H.,Ishihara Y.,Ozaki Y.,Nagashima K.: "Wernicke encephalopathy-like symptoms as an early manifestation of Creutzfeldt-Jakob disease in a chronic alcoholic"J. Neurol. Sci. 163. 192-198 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Murayama J.,Shin R-W,Higuchi J.,Shibuya S.,Muramoto T.,Kitamoto T.: "Interaction of Aluminum with PHFt in Alzheimer's disease neurofibrillary degeneration evidenced by desferrioxamine-assisted chelating autoclave method"Am. J. Pathol. 155. 877-885 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Yamasaki M.,Oyanagi K.,Mori O.,Ohyama M.,Terashi A.,Kitamoto T.,Katayama Y.: "Variant Gerstmann-Straussler syndrome with the P105L prion protein gene mutation: an unusual case with nigral degeneration and widespread neurofibrillary tangles"Acta Neuropathol.. 98. 506-511 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] Shibuya S,Higuchi J,Shin R-W,Tateishi J,Kitamoto T.: "Protective prion protein polymorphisms against sporadic Creutzfeldt-Jakob disease." Lanset. 351. 419 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Shibuya S,Shin R-W,Higuchi J,Tateishi J,Kitamoto T.: "Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease." Ann Neurol. 43. 826-828 (1998)

    • Related Report
      1998 Annual Research Report
  • [Publications] Shimizu S,Hoshi K,Muramoto T,Homma M,Ironside JW,Kuzuhara S,Sato T,Yamamoto T,Kitamoto T.: "Creutzfeldt-Jakob disease with florid plaques after cadaveric dural grafting." Auch.Neurol. (in press). (1998)

    • Related Report
      1998 Annual Research Report

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Published: 1998-04-01   Modified: 2016-04-21  

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