Abstract
Prion diseases are fatal degenerative disorders whose features include the accumulation of abnormal isoform of prion protein (PrPSc), vacuolation, and astrocytosis in the brain. After a prion infection, the cellular isoform of prion protein (PrPC) is converted into PrPSc, resulting in PrPC deficiency and PrPSc accumulation in the brain. These changes are major etiological events, thought to be closely related to the pathogenesis of prion diseases, and used as an index for diagnosis. Studies using recently developed research tools such as transgenic and knockout mice and cell lines have shown that the accumulation of PrPSc is not the sole factor contributing to the clinical onset of prion diseases and that loss-of function of PrPC by depletion leads to neuronal cell loss. Notably, PrPC plays an important role in anti-oxidative defense and its deficiency increases susceptibility to oxidative stress. Furthermore, there is a possible interrelationship between Alzheimers disease and prion disease through loss-of-function of PrPC, which leads to the production of amyloid β. In this review, we introduce research and diagnostic tools for prion diseases and the involvement of loss-of-function of PrPC in the pathogenicity of prion diseases.
Keywords: Prion protein, neurodegenerative disease, PrP-deficient cells, oxidative stress, mitochondria, Alzheimer's disease, loss-of-function
Protein & Peptide Letters
Title: Fundamentals of Prion Diseases and Their Involvement in the Loss of Function of Cellular Prion Protein
Volume: 16 Issue: 3
Author(s): Akikazu Sakudo and Kazuyoshi Ikuta
Affiliation:
Keywords: Prion protein, neurodegenerative disease, PrP-deficient cells, oxidative stress, mitochondria, Alzheimer's disease, loss-of-function
Abstract: Prion diseases are fatal degenerative disorders whose features include the accumulation of abnormal isoform of prion protein (PrPSc), vacuolation, and astrocytosis in the brain. After a prion infection, the cellular isoform of prion protein (PrPC) is converted into PrPSc, resulting in PrPC deficiency and PrPSc accumulation in the brain. These changes are major etiological events, thought to be closely related to the pathogenesis of prion diseases, and used as an index for diagnosis. Studies using recently developed research tools such as transgenic and knockout mice and cell lines have shown that the accumulation of PrPSc is not the sole factor contributing to the clinical onset of prion diseases and that loss-of function of PrPC by depletion leads to neuronal cell loss. Notably, PrPC plays an important role in anti-oxidative defense and its deficiency increases susceptibility to oxidative stress. Furthermore, there is a possible interrelationship between Alzheimers disease and prion disease through loss-of-function of PrPC, which leads to the production of amyloid β. In this review, we introduce research and diagnostic tools for prion diseases and the involvement of loss-of-function of PrPC in the pathogenicity of prion diseases.
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Cite this article as:
Sakudo Akikazu and Ikuta Kazuyoshi, Fundamentals of Prion Diseases and Their Involvement in the Loss of Function of Cellular Prion Protein, Protein & Peptide Letters 2009; 16 (3) . https://dx.doi.org/10.2174/092986609787601732
DOI https://dx.doi.org/10.2174/092986609787601732 |
Print ISSN 0929-8665 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5305 |
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