prion DZ

Question 0

prions

Answer 0

transmissible, resistant to normal sterilization

Question 1

prions cause dz that involves?

Answer 1

CNS: misfolded proteins aggregate in CNS –> causes progressive neurodegeneration –> death

Question 2

diseases that prions cause

Answer 2

1. kuru - “trembling”, transmitted through cannibalism, found in Papa New Guinea
2. Creutzfeldt-Jakob disease (CJD), sporadic, familial, or iatrogenic: transmitted by corneal and dural implants, human GH from cadavers.
   
   • causes a rapidly progressing neurodegenerative disorder, usually ppl >65yo onset. dementia, myoclonus, behavioral changes.
   • new variant CJD: assoc with bovine spongiform encephalopathy (BSE) = mad cow disease,
     
     • met-met of gene PRNP codes for prion protein
3. Fatal familial insomnia
4. Gerstmann-Strausler-Scheinkar ad (cerebellar degeneration–> dementia)