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Biochemistry of Scrapie Pathogenesis

Investigators
Caughey, Byron
Institutions
DHHS/NIH - National Institute of Allergy and Infectious Diseases
Start date
2000
End date
2000
Objective
Transmissible spongiform encephalopathies (TSEs or prion disease) are fatal neurodegenerative diseases such as scrapie, Creutzfeldt-Jakob disease (CJD), BSE and chronic wasting disease (CWD). Our project is aimed at understanding and thwarting the accumulation of PrP-res, the abnormal form of prion protein (PrP) that appears to underlie TSE transmission and pathogenesis.
More information
Using cell culture and cell-free systems we have
  1. Identified lysosomotropic amines, cysteine protease inhibitors and certain new PrP peptide fragments as potentially therapeutic new inhibitors of PrP-res formation,
  2. Described sites of interaction that occur during PrP-res formation,
  3. Described interactions between normal PrP and PrP-res molecules from different species that may control interspecies transmissibilities of TSEs,
  4. Determined effects of the membrane attachment of normal PrP on its conversion to PrP-res,
  5. Assayed the reversibility of PrP-res formation,
  6. Identified heparan sulfate and serum amyloid P component as stimulators of PrP-res formation,
  7. Assessed the ability of PrP-res from CWD-infected deer and elk to induce the conversion of normal PrP from humans, cattle and other species to PrP-res and
  8. Showed that a detergent can induce conformational changes and fibril formation in normal PrP that are reminiscent of PrP-res formation.
Funding Source
Nat'l. Inst. of Allergy and Infectious Diseases
Project number
1Z01AI000580-11
Categories
Bacterial Pathogens
Viruses and Prions
Commodities
Meat, Poultry, Game