PRIONS DISEASE

Question 1

A protein that changes configuration when sticks to an abnormal protein

Answer 1

Prion protein

Question 2

Prions are called proteinaceous infectious particle discovered by

Answer 2

Stanley Prusiner

Question 3

A group of uniformly fatal neurodegenerative diseases caused by the transformation of an endogenous protein, PrP (prion-related protein), into an abnormal conformation called the prion

Answer 3

prion diseases

Question 4

Gene that encodes for prion-related protein

Answer 4

PRNP gene

Question 5

normal cellular prion-related protein

Answer 5

PrPc

Question 6

PrPsc

Answer 6

abnormal infectious form

Question 7

Sc refers to the abnormally shaped PrP found in

Answer 7

scrapie

Question 8

T/F: PrPc and PrPsc have the same identical amino acid sequences (except in genetic prion diseases) but have different three-dimensional structures.

Answer 8

TRUE

Question 9

Current prion models of disease that explain how
prions are transmitted:

Answer 9

Refolding
Seeding

Question 10

Conformational change is kinetically controlled, a high- activation energy barrier preventing spontaneous conversion at detectable rates.

Answer 10

Refolding model

Question 11

Interaction with exogenously introduced PrPSc causes PrPC to undergo an induced conformational change to yield PrPSc → prions accumulate → protein misfolding or refolding

Answer 11

Refolding model

Question 12

spontaneous change happening to the PrPc becoming PrPsc → a very slow process happens where PrPsc attaches to other PrPc → rapidly increases in size similar to the formation of amyloid.

Answer 12

Seeding model

Question 13

______________has been implicated in multi-drug resistance (MDR) of gastric cancer cells,

Answer 13

Octarepeat peptide regions of PrPc

Question 14

★ Peak Age of onset for sCJD:

Answer 14

68 years old

Question 15

“to shake or tremble”

Answer 15

Kuru

Question 16

Transmitted via eating deceased relatives (honored ritual). Occurred first in Fore tribe in Papua New Guinea due to endocannibalism (human-to-human transmission)

Answer 16

Kuru

Question 17

Kuru is associated with heterozygote at codon ________

Answer 17

129 (methionine and valine)

Question 18

cases reported due to cadaveric-derived human pituitary hormones, dura matter grafts, corneal transplants, reuse of cleaned and sterilized EEG depth electrodes directly implanted into the brain, other neurosurgical equipment, blood transfusion

Answer 18

IATROGENIC CREUTZFELDT-JAKOB DISEASE

Question 19

iCJD presents predominantly with _______________

Answer 19

cerebellar dysfunction

Question 20

Most notorious form of acquired human prion disease. Caused by inadvertent ingestion of beef contaminated with BSE or in few cases, blood or blood product transfusion from asymptomatic patients who are vCJD carriers

Answer 20

VARIANT CREUTZFELDT-JAKOB DISEASE

Question 21

A relative unique symptom in vCJD

Answer 21

persistent, painful paresthesia in various parts of the body

Question 22

vCJD brain MRI shows

Answer 22

pulvinar signs

Question 23

Pathognomonic for vCJD

Answer 23

abundant PrPsc deposition (multiple fibrillary PrP plaques surrounded by a halo of spongiform vacuoles known as florid plaques), other PrP plaques and amorphous pericellular and perivascular PrP deposits especially in the cerebellar molecular layer

Question 24

Many of the cases of vCJD were homozygous for ___________

Answer 24

valine at codon129 in PRNP

Question 25

Most affected vCJD cases are

Answer 25

methionine homozygous

Question 26

requires method that will denature proteins

Answer 26

Decontamination

Question 27

highly effective in decontaminating medical supplies and instruments.

Answer 27

Guanidine thiocyanate

Question 28

WHO guidelines preferred method of decontamination

Answer 28

WHO guidelines preferred method: ð Steam sterilization for at least 30 minutes at 132°C in a gravity-displacement sterilizer

If prevacuum sterilizer is used, steam it at 18 mins at 134°C also is effective