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10.1016/j.ajpath.2014.08.005

http://scihub22266oqcxt.onion/10.1016/j.ajpath.2014.08.005
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suck abstract from ncbi


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pmid25280631
      Am+J+Pathol 2014 ; 184 (12 ): 3299-307
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  • Uptake and degradation of protease-sensitive and -resistant forms of abnormal human prion protein aggregates by human astrocytes #MMPMID25280631
  • Choi YP ; Head MW ; Ironside JW ; Priola SA
  • Am J Pathol 2014[Dec]; 184 (12 ): 3299-307 PMID25280631 show ga
  • Sporadic Creutzfeldt-Jakob disease is the most common of the human prion diseases, a group of rare, transmissible, and fatal neurologic diseases associated with the accumulation of an abnormal form (PrP(Sc)) of the host prion protein. In sporadic Creutzfeldt-Jakob disease, disease-associated PrP(Sc) is present not only as an aggregated, protease-resistant form but also as an aggregated protease-sensitive form (sPrP(Sc)). Although evidence suggests that sPrP(Sc) may play a role in prion pathogenesis, little is known about how it interacts with cells during prion infection. Here, we show that protease-sensitive abnormal PrP aggregates derived from patients with sporadic Creutzfeldt-Jakob disease are taken up and degraded by immortalized human astrocytes similarly to abnormal PrP aggregates that are resistant to proteases. Our data suggest that relative proteinase K resistance does not significantly influence the astrocyte's ability to degrade PrP(Sc). Furthermore, the cell does not appear to distinguish between sPrP(Sc) and protease-resistant PrP(Sc), suggesting that sPrP(Sc) could contribute to prion infection.
  • |Animals [MESH]
  • |Astrocytes/cytology/*metabolism [MESH]
  • |Brain/metabolism/*pathology [MESH]
  • |Creutzfeldt-Jakob Syndrome/*metabolism [MESH]
  • |Endopeptidase K/chemistry [MESH]
  • |Epitopes/chemistry [MESH]
  • |Fibroblasts/metabolism [MESH]
  • |Humans [MESH]
  • |Immunoprecipitation [MESH]
  • |Mice [MESH]
  • |Microscopy, Fluorescence [MESH]
  • |Peptide Hydrolases/*chemistry [MESH]
  • |Phosphotungstic Acid/chemistry [MESH]
  • |PrPC Proteins/*metabolism [MESH]
  • |PrPSc Proteins/metabolism [MESH]
  • |Prion Diseases/metabolism [MESH]


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