The importance of the disulfide bond in prion protein conversion

Neuroreport. 1998 Aug 3;9(11):2457-61. doi: 10.1097/00001756-199808030-00006.

Abstract

The conversion of normal, protease sensitive prion protein (PrP-sen) to the abnormal protease-resistant form (PrP-res) is of central importance in the pathogenesis of scrapie and other transmissible spongiform encephalopathies. In the present study, the effects of reduction of the disulfide bond on the PrP-sen to PrP-res conversion in a cell-free system were examined. The addition of the disulfide reducing agent dithiothreitol inhibited the cell-free conversion reaction with an IC50 of 2-2.5 mM. Separate pretreatment of either PrP-sen or PrP-res with dithiothreitol and an alkylating agent also inhibited the conversion reaction. Results of this study show that preservation of the disulfide bond is important in the conversion of PrP-sen to PrP-res.

MeSH terms

  • Alkylation
  • Animals
  • Ascorbic Acid / chemistry
  • Autoradiography
  • Cell-Free System
  • Cricetinae
  • Disulfides / chemistry
  • Dithionite / chemistry
  • Dithiothreitol / chemistry
  • Endopeptidase K / chemistry
  • Mesocricetus
  • Prions / chemistry*
  • Protein Conformation
  • Reducing Agents / chemistry
  • Sulfhydryl Reagents / chemistry

Substances

  • Disulfides
  • Prions
  • Reducing Agents
  • Sulfhydryl Reagents
  • Dithionite
  • Endopeptidase K
  • Ascorbic Acid
  • Dithiothreitol