Emergence of two prion subtypes in ovine PrP transgenic mice infected with human MM2-cortical Creutzfeldt-Jakob disease prions

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• 作者：Jérôme Chapuis ; Mohammed Moudjou ; Fabienne Reine…
• 关键词：Prion ; CJD ; Mutation ; Sporadic ; Transgenic mice
• 刊名：Acta Neuropathologica Communications
• 出版年：2016
• 出版时间：December 2016
• 年：2016
• 卷：4
• 期：1
• 全文大小：4,279 KB
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• 作者单位：Jérôme Chapuis (1)
  Mohammed Moudjou (1)
  Fabienne Reine (1)
  Laetitia Herzog (1)
  Emilie Jaumain (1)
  Céline Chapuis (1)
  Isabelle Quadrio (2) (3)
  Jacques Boulliat (4)
  Armand Perret-Liaudet (2) (3)
  Michel Dron (1)
  Hubert Laude (1)
  Human Rezaei (1)
  Vincent Béringue (1)
  
  1. INRA (Institut National de la Recherche Agronomique), UR892, Virologie Immunologie Moléculaires, F-78350, Jouy-en-Josas, France
  2. Neurobiology Laboratory, Biochemistry and Molecular Biology Department, Hôpitaux de Lyon, Lyon, France
  3. University of Lyon 1, CNRS UMR5292, INSERM U1028, BioRan, Lyon, France
  4. Neurology Department, Centre Hospitalo-Universitaire, Bourg en Bresse, France
  
• 刊物主题：Neurosciences;
• 出版者：BioMed Central
• ISSN：2051-5960

文摘

Introduction Mammalian prions are proteinaceous pathogens responsible for a broad range of fatal neurodegenerative diseases in humans and animals. These diseases can occur spontaneously, such as Creutzfeldt-Jakob disease (CJD) in humans, or be acquired or inherited. Prions are primarily formed of macromolecular assemblies of the disease-associated prion protein PrPSc, a misfolded isoform of the host-encoded prion protein PrPC. Within defined host-species, prions can exist as conformational variants or strains. Based on both the M/V polymorphism at codon 129 of PrP and the electrophoretic signature of PrPSc in the brain, sporadic CJD is classified in different subtypes, which may encode different strains. A transmission barrier, the mechanism of which remains unknown, limits prion cross-species propagation. To adapt to the new host, prions have the capacity to ‘mutate’ conformationally, leading to the emergence of a variant with new biological properties. Here, we transmitted experimentally one rare subtype of human CJD, designated cortical MM2 (129 MM with type 2 PrPSc), to transgenic mice overexpressing either human or the VRQ allele of ovine PrPC.