Pathology : Neuroanatomical Distribution of Disease-Associated Prion Protein in Cases of Bovine Spongiform Encephalopathy Detected by Fallen Stock Surveillance in Japan

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  • OKADA Hiroyuki
    Prion Disease Research Center, National Institute of Animal Health
  • IWAMARU Yoshihumi
    Prion Disease Research Center, National Institute of Animal Health
  • IMAMURA Morikazu
    Prion Disease Research Center, National Institute of Animal Health
  • MASUJIN Kentaro
    Prion Disease Research Center, National Institute of Animal Health
  • MATSUURA Yuichi
    Prion Disease Research Center, National Institute of Animal Health
  • SHIMIZU Yoshihisa
    Prion Disease Research Center, National Institute of Animal Health
  • KASAI Kazuo
    Prion Disease Research Center, National Institute of Animal Health
  • TAKATA Masuhiro
    Prion Disease Research Center, National Institute of Animal Health
  • FUKUDA Shigeo
    Hokkaido Animal Research Center, Hokkaido Research Organization
  • NIKAIDO Satoshi
    Hokkaido Animal Research Center, Hokkaido Research Organization
  • FUJII Kei
    Hokkaido Animal Research Center, Hokkaido Research Organization
  • ONOE Sadao
    Hokkaido Animal Research Center, Hokkaido Research Organization
  • MOHRI Shirou
    Prion Disease Research Center, National Institute of Animal Health
  • YOKOYAMA Takashi
    Prion Disease Research Center, National Institute of Animal Health

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  • Neuroanatomical Distribution of Disease-Associated Prion Protein in Cases of Bovine Spongiform Encephalopathy Detected by Fallen Stock Surveillance in Japan

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Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disorder of cattle characterized by accumulation of the disease-associated prion protein (PrPSc) in the central nervous system (CNS). The immunohistochemical patterns and distribution of PrPSc were investigated in the CNS, brains, and spinal cords of 7 naturally occurring BSE cases confirmed by the fallen stock surveillance program in Japan. No animals showed characteristic clinical signs of the disease. Coronal slices of 14 different brain areas in each case were immunohistochemically analyzed using an anti-prion protein antibody. Immunolabeled PrPSc deposition was widely observed throughout each brain and spinal cord. Intense PrPSc deposition was greater in the thalamus, brainstem, and spinal cord of the gray matter than in the neocortices. The topographical distribution pattern and severity of PrPSc accumulation were mapped and plotted as immunohistochemical profiles of the different brain areas along the caudal-rostral axis of the brain. The distribution pattern and severity of the immunolabeled PrPSc in the CNS were almost the same among the 7 cases analyzed, suggesting that the naturally occurring cases in this study were at the preclinical stage of the disease. Immunohistochemical mapping of the PrPSc deposits will be used to clarify the different stages of BSE in cattle.<br>

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