16- prion related diseases

prion is a protein particle which is infectious without any nucleic acids or virus like structure or properties. the diseases caused by prions are :

• In animals:
  • Scrapie in sheep and goats^[26]
  • Bovine spongiform encephalopathy (BSE) in cattle (known as mad cow disease)^[26]
  • Transmissible mink encephalopathy (TME) in mink^[26]
  • Chronic wasting disease (CWD) in elk and mule deer^[26]
  • Feline spongiform encephalopathy in cats^[26]
  • Exotic ungulate encephalopathy (EUE) in nyala, oryx and greater kudu^[26]
  • Spongiform encephalopathy of the ostrich^[27]
• In humans:
  • Creutzfeldt-Jakob disease (CJD)^[26] and its varieties: iatrogenic Creutzfeldt-Jakob disease (iCJD), variant Creutzfeldt-Jakob disease (vCJD), familial Creutzfeldt-Jakob disease (fCJD), and sporadic Creutzfeldt-Jakob disease (sCJD)
  • Gerstmann-Sträussler-Scheinker syndrome (GSS)^[26]
  • Fatal familial insomnia (fFI)^[26]
  • Sporadic fatal insomnia (sFI)^[28]
  • Kuru^[26]
  • Alpers syndrome^[27]

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Disease	Host	Mechanism
Kuru	Human	Cannibalism
Sporadic CJD	Human	Spontaneous PrPC to PrPSc conversion or somatic mutation
Iatrogenic CJD	Human	Infection from prion-containing material, eg, dura mater, electrode
Familial CJD	Human	Mutations in the PrP gene
vCJD	Human	Infection from BSE
GSS	Human	Mutations in the PrP gene
FFI	Human	D178N mutation in the PrP gene, with M129 polymorphism
Sporadic fatal insomnia	Human	Spontaneous PrPC to PrPSc conversion or somatic mutation
Scrapie	Sheep	Infection in susceptible sheep
BSE	Cattle	Infection from contaminated food
TME	Mink	Infection from sheep or cattle in food
CWD	Mule, deer, elk	Unclear
Feline spongiform encephalopathy	Cats	Infection from contaminated food
Exotic ungulate encephalopathy	Nyala, oryx, kudu	Infection from contaminated food

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