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From BSE t o Prion

From BSE t o Prion. Prion: An infectious proteinaceous particle of nonnucleic acid composition. Bovine spongiform encephalopathy (BSE ) mad-cow diseas e. Prion protein (PrP) : from PrP C to PrP S c. Same amino acid sequences but different structures. PrP S c (scrapie prion protein)

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From BSE t o Prion

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  1. From BSE to Prion Prion: An infectious proteinaceous particle of nonnucleic acid composition

  2. Bovine spongiform encephalopathy (BSE) mad-cow disease

  3. Prion protein (PrP): from PrPC to PrPSc Same amino acid sequences but different structures PrPSc (scrapie prion protein) 43% β- sheet and 30% α-helix Insoluble Resistant to protease K Pathogenic PrPC (cellular prion protein) Mainly α-helix Soluble Sensitive to protease K Has normal functions, not pathogenic

  4. Prions aggregate extracellularly within the central nervous system to form plaques known as amyloid, which disrupt the normal tissue structure Strong resistance to autoclave, UV, regular disinfectants. Standard autoclave (121oC, 20 min) does not inactivate it. Needs 134oC, 2h

  5. Transmissible SpongiformEncephalopathies (Prion Diseases) • Human – Kuru disease – Creutzfeldt--Jacob disease (CJD) – variant Creutzfeldt--Jacob disease (vCJD) • Animals Fore child in Papua New Guinea with Kuru – Scrapie of sheep and goats – Bovine spongiform encephalopathy (BSE, mad cow) Progressive neurodegenerative disease

  6. The characteristics of Prion diseases normal

  7. Diagnosis and Prevention • Immunoblotting assay (Western blot): Treat tissue with proteinase K to digest PrPC, and then analyze with Western blot for the presence of PrPSc • Immunohistochemstry: Also treat with proteinase K first • Genetic analysis: For inherited mutations in the PrP gene. • Prevention: quarantine of animals and complete disinfection of medical devices. No treatment.

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