Prions

Question 1

Prions are infectious proteins found in both ______

Answer 1

mammals and fungi

Question 2

Prions are devoid of ______ that directs the synthesis of their progeny

Answer 2

nucleic acid genome

Question 3

Prions multiply by forcing the precursor protein to adopt a ____

Answer 3

alternative conformation

Question 4

Which system do the prions accumulate where they cause dysfunction and fatal degeneration?

Answer 4

CNS

Question 5

Infectious human prion diseases are transmitted to others by _______

Answer 5

ritualistic cannibalism and iatrogenic

Question 6

Organs that support PrPSc formation

Answer 6

Neurons,, Myocytes, Follicular dendritic cells, B lymphocytes

Question 7

Prion formation happens post _____

Answer 7

transcriptionally

Question 8

How many copies of octarepeat sequence in humans?

Answer 8

5

Question 9

In PrPc structure, which is greater in quantity, alpha helix or beta sheets

Answer 9

Alpha helix

Question 10

Transmissible Spongiform Encephalopathies is distinguishable by: ____

Answer 10

○ Long incubation period
○ Characteristic spongiform changes associated with neuronal loss
○ Failure to induce inflammatory response

Question 11

Enumerate the selected prion diseases in humans

Answer 11

• Classic CREUTZFELDT-JAKOB DISEASE
• VARIANT CREUTZFELDT-JAKOB DISEASE (vCJD)
• GERSTMANN-STRAUSSLER-SCHEINKER
• FATAL INSOMNIA

Question 12

Enumerate prion diseases in animals

Answer 12

• BOVINE SPONGIFORM ENCEPHALOPATHY
• CHRONIC WASTING DISEASE
• MOLECULAR PATHOGENESIS

Question 13

Surrogate Markers of Prions

Answer 13

• mRNA transcript of erythroid differentiation-related factor(EDRF) in blood
• Stress protein 14-3-3 in CSF
• Protease-resistant PrP in urine
• Capture ELISA of PrPSc in blood

Question 14

Diagnostic tests for BSE

Answer 14

Western blotting and ELISA tests for rPrPSc on the brain stems from cattles.

Question 15

C-terminal sequence beckoning addition of ____

Answer 15

GPI anchor

Question 16

Two ____ side chains linked to large oligosaccharides

Answer 16

Asparagine (Asn)

Question 17

Sporadic CJD can be classified as ____

Answer 17

Definite, Probable, or Possible

Question 18

vCJD has the same agent as which prion disease?

Answer 18

Mad cow disease

Question 19

Definitive case of vCJD has numerous widespread ______

Answer 19

kuru-type amyloid plaques

Question 20

GERSTMANN-STRAUSSLER-SCHEINKER is _____ category of prion disease

Answer 20

Heritable

Question 21

GSS is linked to ____ mutation of the PrP gene

Answer 21

P102L

Question 22

Fatal insomnia is associated with _____ mutation

Answer 22

D178N mutation

Question 23

Chronic wasting disease is the only prion disease known in _____

Answer 23

free-ranging animals