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PRIONS
What is a Prion?
• Infectious agent that is
composed primarily of protein
• Polypeptide = amino acid chain
= protein
• Can be one or many
polypeptides
What do they do?
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Cause memory changes
Impair brain function
Personality changes
Decline in intellectual function
(dementia)
• Problems with movement that worsen
over time
Prion Diseases
• Human Prion Diseases
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CJD = Creutzfekdt-Jakob Disease
vCJD = Variant Creutzfelt-Jakob Disease
GSS = Gerstmann-Straussler-Scheinker Syndrome
FFI = Fatal Familial Insomnia
Kuru = New Guinea
• Animal Prion Diseases
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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
Who Gets Prion Diseases?
• 1/per million affected worldwide
each year
• Approximately 300 cases annually
in the USA
• Rarely genetic
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.
• http://www.youtube.com/watch?v=ujK7KiLt
0s8 (NOVA) 52 mins: Published on Nov 18, 2013
• http://www.youtube.com/watch?v=pqhpVp
afjmk (Prions: short ~ 1:40)
• http://www.youtube.com/watch?v=VgB9sg
5dy8w MAD COW: USA - Published on Apr 24,
2012
• Kuru:
https://www.youtube.com/watch?v=vw_tCl
cS6To (52:10)
• http://www.cdc.gov/ncidod/teachers_tools/
virus_encounters.htm