Comparison of protease-resistant prion protein inhibitors in cell cultures infected with two strains of mouse and sheep scrapie

Neurosci Lett. 2005 Nov 11;388(2):106-11. doi: 10.1016/j.neulet.2005.06.053.

Abstract

The transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases. A primary therapeutic target for TSE intervention has been a protease-resistant form of prion protein known as PrP(Sc) or PrP-res. In vitro testing of mouse scrapie-infected cell cultures has identified many PrP-res inhibitors that also have activity in vivo. Here we identify 32 new inhibitors of two strains of mouse scrapie PrP-res. Furthermore, to investigate the species-specificity of these and other PrP-res inhibitors, we have developed a high-throughput cell culture assay based on Rov9 cells chronically-infected with sheep scrapie. Of 32 inhibitors of murine PrP-res that were also tested in the Rov9 cells, only six showed inhibitory activity against sheep PrP-res. The three most potent inhibitors of both murine and ovine PrP-res formation (with 50% inhibition at < or =5 microM) were tannic acid, pentosan polysulfate and Fe(III) deuteroporphyrin 2,4-bisethyleneglycol. The latter two have anti-mouse scrapie activity in vivo. These results identify new inhibitors of murine and ovine PrP-res formation and reinforce the idea that compounds effective against PrP-res from one species or strain cannot be assumed to be active against others.

Publication types

  • Comparative Study
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Animals
  • Cell Line
  • Deuteroporphyrins / pharmacology
  • Enzyme Inhibitors / pharmacology
  • Epithelial Cells / cytology
  • Ferric Compounds / pharmacology
  • Mice
  • Pentosan Sulfuric Polyester / pharmacology
  • PrPSc Proteins / antagonists & inhibitors*
  • PrPSc Proteins / metabolism
  • Rabbits
  • Scrapie / drug therapy*
  • Scrapie / metabolism*
  • Sheep
  • Species Specificity
  • Tannins / pharmacology*

Substances

  • Deuteroporphyrins
  • Enzyme Inhibitors
  • Ferric Compounds
  • PrPSc Proteins
  • Tannins
  • deuteroporphyrin IX 2,4-bis(ethylene glycol)iron III
  • Pentosan Sulfuric Polyester