Prions

Question 1

what are prions

Answer 1

infectious proteins (NOT nucleic acids)
*PROteinaceous and INfectious

Question 2

what disease do prions cause

Answer 2

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES:
-fatal, neurodegenerative disorders

Question 3

clinical course of prion diseases

Answer 3

slow progression of neurologic degenerative disease

Question 4

examples of prion diseases

Answer 4

-Creutzfeld-Jakob disease
-fatal familial insomnia
-Gerstmann-Straussler syndrome
-Kuru
-Variant Creutzfeld-Jakob disease

Question 5

structure of prions

Answer 5

pathogenic prion is a conformational isoform of a normal host protein, PrPc
*PrPc predominantly found on outer surfaces of neurons, GPI anchor

Question 6

prion replication

Answer 6

when introduced into an organism, the abnormal conformer causes CONVERSION of NORMAL PrPc into the PATHOGENIC conformation

Question 7

prion disease epidemiology

Answer 7

rare, predominantly GENETIC but can be from consumption

Question 8

prion disease clinical presentation

Answer 8

cerebellar ataxia, ultimately DEATH, spongiform appearance of brain

Question 9

prion disease testing

Answer 9

genetic testing (if inherited disease form)

Question 10

prion disease treatment

Answer 10

NONE; these diseases are invariably FATAL

Question 11

concern about prion diseases

Answer 11

concern that “chronic wasting disease” of deer, elk, and moose could infect hunters