Prion Diseases Flashcards
T/F:
Abnormal prion proteins differ from normal prions in that they have different amino acid sequences and different folding patterns.
False
Folding pattern only
Which of the following is MISMATCHED ?
A. PrPc: alpha pleated sheet, alpha helix
B. PrPsen: alpha pleated sheet, alpha helix
C. PrPsc: beta pleated sheet, beta helix
D. PrPsen: beta pleated sheet, beta helix
D.
sen is same as c
sc=scrapie!
Which of the following are infectious prions inactivated by?
A. Formaldehyde B. Radiation C. Boiling D. Protease enzyme digestion E. Autoclaving at high temps for extended periods
E.
What is the most common form of spongiform encephalopathy?
A Familial
B sporadic
C. Infectious
D. Uknown
B Sporadic
This is the one that occurs randomly in aging brains, more applicable in humans
T/F:
Infectious/Acquired spongiform encephalopathy can be transmitted by use of purified hormones
True!
Weird. Ok.
Histopathology for Spongiform Encephalopathy will have :
A. Perivascular cuffing of neurons
B. Extensive changes indicative of inflammation
C Vacuolization of grey matter
D. Astrocytosis (astroglial hypertrophy and hyperplasia)
E. Neuron loss
F. C, D, E only
F
T/F:
Infectious prion disease is so progressive and devastating because it produces massive inflammation caused by overactivation of the host immune response
False
NO inflammation!
No host immune response!
Insidious!
For Scrapie, what is NOT true?
A. Affects sheep
B. First sign often pruritis
C. sAsA or sApA shorter incubation period, die sooner
D. pApA: longer incubation period, longer to die
E. Takes several years to die after developing signs
E.
Takes 6 wks to 5 months after clinical signs start before sheep dies
What is the GOLD STANDARD for testing sheep for Scrapie?
A. ELISA B. PCR C. tonsillar biopsy and IHC staining D. 3rd eyelid test E. C and D
E
T/F:
Mad Cow Disease will have characteristic fibrils when seen on electron microscopy.
False
SCRAPIE
T/F:
Scrapie is a reportable disease in the U.S. There is a genetic monitoring program by the USDA in which flocks are genotyped and susceptible genotypes are removed- flocks are under surveillance for 5 years.
True
T/F:
Mad Cow Disease is similar to Scrapie in sheep except there is no breed or genetic susceptibility, incubation period is not variable based on genetic susceptibility, humans are more known to be able to be hosts, and pruritis is NOT a sign.
True
