Prion protein scrapie and the normal cellular prion protein

被引:39
|
作者
Atkinson, Caroline J. [1 ]
Zhang, Kai [1 ]
Munn, Alan L. [2 ,3 ]
Wiegmans, Adrian [4 ]
Wei, Ming Q. [1 ]
机构
[1] Griffith Univ, Div Mol & Gene Therapies, Menzies Hlth Inst, Gold Coast, Qld, Australia
[2] Griffith Univ, Menzies Hlth Inst Queensland, Mol Basis Dis Program, Lab Yeast Cell Biol, Gold Coast, Qld, Australia
[3] Griffith Univ, Sch Med Sci, Gold Coast, Qld, Australia
[4] QIMR Berghofer Med Res Inst, Tumour Microenvironm Lab, Herston, Qld, Australia
基金
澳大利亚研究理事会;
关键词
Creutzfeldt-Jakob disease; bovine spongiform encephalopathy; Alzheimer's disease; infectious protein; prion treatment; PRNP; CREUTZFELDT-JAKOB-DISEASE; DEPRESSIVE-LIKE BEHAVIOR; STRAUSSLER-SCHEINKER-SYNDROME; COPPER-BINDING PROTEIN; COGNITIVE DEFICITS; BLOOD-TRANSFUSION; COLORECTAL-CANCER; ALZHEIMER-DISEASE; MOLECULAR ASPECTS; DENDRITIC CELLS;
D O I
10.1080/19336896.2015.1110293
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Prions are infectious proteins and over the past few decades, some prions have become renowned for their causative role in several neurodegenerative diseases in animals and humans. Since their discovery, the mechanisms and mode of transmission and molecular structure of prions have begun to be established. There is, however, still much to be elucidated about prion diseases, including the development of potential therapeutic strategies for treatment. The significance of prion disease is discussed here, including the categories of human and animal prion diseases, disease transmission, disease progression and the development of symptoms and potential future strategies for treatment. Furthermore, the structure and function of the normal cellular prion protein (PrPC) and its importance in not only in prion disease development, but also in diseases such as cancer and Alzheimer's disease will also be discussed.
引用
收藏
页码:63 / 82
页数:20
相关论文
共 50 条
  • [1] Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation
    Kaneko, K
    Zulianello, L
    Scott, M
    Cooper, CM
    Wallace, AC
    James, TL
    Cohen, FE
    Prusiner, SB
    PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1997, 94 (19) : 10069 - 10074
  • [2] Association of prion protein genotype and scrapie prion protein type with cellular prion protein charge isoform profiles in cerebrospinal fluid of humans with sporadic or familial prion diseases
    Schmitz, Matthias
    Luellmann, Katharina
    Zafar, Saima
    Ebert, Elisabeth
    Wohlhage, Marie
    Oikonomou, Panteleimon
    Schlomm, Markus
    Mitrova, Eva
    Beekes, Michael
    Zerr, Inga
    NEUROBIOLOGY OF AGING, 2014, 35 (05) : 1177 - 1188
  • [3] Ultrastructure and pathology of prion protein amyloid accumulation and cellular damage in extraneural tissues of scrapie-infected transgenic mice expressing anchorless prion protein
    Race, Brent
    Jeffrey, Martin
    McGovern, Gillian
    Dorward, David
    Chesebro, Bruce
    PRION, 2017, 11 (04) : 234 - 248
  • [4] Dimerization of the cellular prion protein inhibits propagation of scrapie prions
    Engelke, Anna D.
    Gonsberg, Anika
    Thapa, Simrika
    Jung, Sebastian
    Ulbrich, Sarah
    Seidel, Ralf
    Basu, Shaon
    Multhaup, Gerd
    Baier, Michael
    Engelhard, Martin
    Schaetzl, Hermann M.
    Winklhofer, Konstanze F.
    Tatzelt, Joerg
    JOURNAL OF BIOLOGICAL CHEMISTRY, 2018, 293 (21) : 8020 - 8031
  • [5] The cellular prion protein beyond prion diseases
    Manni, Giorgia
    Lewis, Victoria
    Senesi, Matteo
    Spagnolli, Giovanni
    Fallarino, Francesca
    Collins, Steven J.
    Mouillet-Richard, Sophie
    Biasini, Emiliano
    SWISS MEDICAL WEEKLY, 2020, 150
  • [6] Fundamentals of Prion Diseases and Their Involvement in the Loss of Function of Cellular Prion Protein
    Sakudo, Akikazu
    Ikuta, Kazuyoshi
    PROTEIN AND PEPTIDE LETTERS, 2009, 16 (03) : 217 - 229
  • [7] Altered trafficking of abnormal prion protein in atypical scrapie: prion protein accumulation in oligodendroglial inner mesaxons
    Jeffrey, M.
    Gonzalez, L.
    Simmons, M. M.
    Hunter, N.
    Martin, S.
    McGovern, G.
    NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 2017, 43 (03) : 215 - 226
  • [8] Sensitive detection of scrapie prion protein in soil
    Nagaoka, Kazunari
    Yoshioka, Miyako
    Shimozaki, Noriko
    Yamamura, Tomoaki
    Murayama, Yuichi
    Yokoyama, Takashi
    Mohri, Shirou
    BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS, 2010, 397 (03) : 626 - 630
  • [9] Insoluble cellular prion protein and its association with prion and Alzheimer diseases
    Zou, Wen-Quan
    Zhou, Xiaochen
    Yuan, Jue
    Xiao, Xiangzhu
    PRION, 2011, 5 (03) : 172 - 178
  • [10] Prion Protein Functions and Dysfunction in Prion Diseases
    Sakudo, Akikazu
    Ikuta, Kazuyoshi
    CURRENT MEDICINAL CHEMISTRY, 2009, 16 (03) : 380 - 389