Prion Diseases

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Prion Diseases
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Plans

• Introduction
• Reasons
• Definitions of Prions disease
• What is  Piron ?
• How it woks? What it implicates?
• Blabla (exemples)

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Introduction
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Definitions of Prion Diseases
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What is  Prion  ?

• History

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What is  Prion  ?

• Prion Proteinaceous Infectious Only particule,
  coined by Stanley Prusiner in 1982 (Nobel Prize
  in Physiology or Medecine in 1997)
• Prion is neither Bacterial, Fungal nor Viral,
  and contains no genetic material.
• Its a small proteinaceous infectious
  disease-causing agent.

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What is  Prion  ?

• PrP (Prion Protein, or Protease-Resistant
  Protein).
• This PrP, also known as CD230 (Cluster of
  Differentiation 230) is the only known prion
  protein in animals.
• In humans, the PrP is encoded by PRNP gene (PRioN
  Protein)
• Predominant in the Nervous System, but present in
  many other tissus all over the body.

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What is  Prion  ?

• PrP can exist in

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What is  Prion  ?
Reminders How Proteins are made from DNA.
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What is  Prion  ?
Reminders Proteins have different levels of
structural organization.
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What is  Prion  ?

• Reponsible for several degenerative brain
  diseases, in animals, and humans.
• In Animals
• Bovine Spongiform Encephalopathy (BSE) (Feline
  Ungulate), Scrapie, Chronic Wasting Diseases
  (CWD), Transmissible Mink Encephalopathy.
• In Humans
• Creutzfeldt-Jakob Disease (CJD) and Variant
  (vCJD), Gerstmann-Straussler-Scheinker Syndrome
  (GSSS), Fatal Familial Insomnia (FFI), and Kuru