中国神经再生研究(英文版) ›› 2021, Vol. 16 ›› Issue (6): 1216-1217.doi: 10.4103/1673-5374.300444

• 观点:神经损伤修复保护与再生 • 上一篇    下一篇

朊病毒蛋白在髓磷脂维持中的作用:山羊怎么说?

  

  • 出版日期:2021-06-15 发布日期:2020-12-31

Prion protein in myelin maintenance:  what does the goat say?

Fredrik S. Skedsmo, Arild Espenes, Michael A. Tranulis*   

  1. Department of Preclinical Sciences and Pathology, Norwegian University of Life Sciences, Oslo, Norway
  • Online:2021-06-15 Published:2020-12-31
  • Contact: Michael A. Tranulis, PhD, Michael.Tranulis@nmbu.no.
  • Supported by:
    This work was supported by grants from the Norwegian University of Life Sciences and The Research Council of Norway (227386/E40).

摘要: https://orcid.org/0000-0002-9222-8509 (Michael A. Tranulis)

Abstract: The cellular prion protein PrPC has been extensively studied because it can adopt a pathogenic three-dimensional conformation that causes rare, but invariably fatal, neurodegenerative prion diseases in humans and other mammals. The disease-causing conformer of the protein is called PrPSc, of which oligomeric aggregates constitute prion agents that can bind to, and convert further, PrPC molecules into PrPSc (Prusiner, 1998). Thus, in the poorly understood process of prion propagation, there is transfer of biological information encoded solely by protein conformation. Prions can spread within a tissue secondary to a spontaneous conversion of PrPC to PrPSc or upon transmission of prion agents between individuals.