1. PRIONS

2. What is a Prion?
Infectious agent that is composed primarily of protein
Polypeptide = amino acid chain = protein
Can be one or many polypeptides

3. What do they do? Cause memory changes Impair brain function
Personality changes
Decline in intellectual function (dementia)
Problems with movement that worsen over time

4. Prion Diseases Human Prion Diseases Animal Prion Diseases
CJD = Creutzfekdt-Jakob Disease
vCJD = Variant Creutzfelt-Jakob Disease
GSS = Gerstmann-Straussler-Scheinker Syndrome
FFI = Fatal Familial Insomnia
Kuru = New Guinea
Animal Prion Diseases
BSE = Bovine Spongiform Encephalopathy = Cow = “Mad Cow”
CWD = Chronic Wasting Disease = Elk, Deer, Moose
Scrapie = Sheep
TME = Transmissible mink encephalopathy
Feline spongiform encephalopathy
Ungulate spongiform encephalopathy

5. Who Gets Prion Diseases?
1/per million affected worldwide each year
Approximately 300 cases annually in the USA
Rarely genetic

13. Is There a Cure?
Currently, there is no cure for any prion disease
Prions cannot be destroyed through cooking, heating, radiation, bleach, auto-claving, etc.

14. http://www. youtube. com/watch
(NOVA) 52 mins: Published on Nov 18, 2013
(Prions: short ~ 1:40)
MAD COW: USA - Published on Apr 24, 2012

15. Kuru: https://www.youtube.com/watch?v=vw_tClcS6To (52:10)