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Accumulation of Abnormal Prion Protein in Non-Central Nervous System Organs
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| Abstract | Human prion diseases, including sporadic Creutzfeldt-Jakob disease, hereditary prion diseases, and iatrogenic Creutzfeldt-Jakob diseases, are fetal neurodegenerative disorders. Prion diseases are char...acterized 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 experiencesshow more |
| Table of Contents | はじめに 1．正常型プリオン蛋白の機能と臓器・細胞局在 2. 異常型プリオン蛋白の伝播様式に関して 3. 全身臓器に沈着する異常型プリオン蛋白(PrPSc) ゲルストマン・ストロイスラー・シャインカー病(GSS) プリオン蛋白コドン178番2塩基欠失遺伝性プリオン病(178変異プリオン病) おわりに 謝辞 |
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| Created Date | 2018.10.25 |
| Modified Date | 2021.03.03 |