全身臓器に沈着する異常型プリオン蛋白

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  • 本田 裕之
    九州大学大学院医学研究院神経病理学分野
  • 岩城 徹
    九州大学大学院医学研究院神経病理学分野

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タイトル別名
  • Accumulation of Abnormal Prion Protein in Non-Central Nervous System Organs
  • ゼンシン ゾウキ ニ チンチャク スル イジョウガタ プリオン タンパク

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抄録

Human prion diseases, including sporadic Creutzfeldt-Jakob disease, hereditary prion diseases, and iatrogenic Creutzfeldt-Jakob diseases, are fetal neurodegenerative disorders. Prion diseases are characterized by the conversion of a normal cellular prion protein isoform into an abnormal pathogenic prion isoform (PrPSc). PrPSc contains high beta sheet content with resistance to proteinase-K. Previously, PrPSc has been considered to be detected only in the central nervous system in prion diseases. However, recently, some reports revealed accumulation of PrPSc in non-central nervous system organs, such as peripheral nerves and muscles, tonsil, lymph nodes, spleen, and pancreas. In this paper, we describe PrPSc deposited in non-central nervous system, including our experiences

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