Prions

Question 1

What is BSE called when it is transmitted to humans

Answer 1

Creutzfeldt-Jakob Disease (CJD)

Question 2

When was the practice of feeding agricultural waste to animals banned

Answer 2

1988

Question 3

Prion definition

Answer 3

Infectious agents made of protein material that can fold multiple abstract ways and at least one of which is transmissible to other prion proteins

Question 4

Prions composed of what protein are the cause of TSE

Answer 4

Prion protein PrP

Question 5

How do prions cause disease

Answer 5

Converting PrPC into aggregation-prone PrPSc

Question 6

Strain

Answer 6

A defined prion population isolated from one specific animal

Question 7

Type

Answer 7

Refers to a combo of biochemical parameters (size of unglycosylated PrPSc fragment after proteinase K partial digestion) that are independent from the host

Question 8

Common characteristics of TSE disease

Answer 8

Long incubation periods, illness of weeks to months, PrPTSE accumulation in brain and other tissues, pathological changes confined to CNS, absence of immune response

Question 9

Scrapie affects…

Answer 9

Sheep and goats

Question 10

Chronic wasting disease affects…

Answer 10

Cervids

Question 11

Transmissible mink encephalopathy affects…

Answer 11

Mink

Question 12

Kuru affects…

Answer 12

Humans (historical)

Question 13

Different strains of TSE cause what conditions

Answer 13

Scrapie, chronic wasting disease, TME, kuru

Question 14

What is the incubation period of CWD

Answer 14

2-4 years

Question 15

Where can PrPSc be found in CWD

Answer 15

Extraneural tissues, body fluids, and excreta (horizontal transmission is possible, one of the most contagious)

Question 16

What is unique about CWD transmission

Answer 16

Indirect transmission occurs via CWD prion deposition into environment from urine, feces and saliva

Question 17

How is BSE thought to arise in cattle

Answer 17

Genetic mutation

Question 18

What is needed to inactive the BSE protein

Answer 18

Immersion in 1N NaOH or a dry heat at 1000C (very stable protein)

Question 19

Symptoms of BSE

Answer 19

Degeneration of nervous system, changes in temperament, abnormalities in posture, nervousness and aggression, incoordination, tremors, hard to stand

Question 20

What is the incubation of BSE

Answer 20

2-8 years

Question 21

How long do clinical symptoms of BSE last

Answer 21

2 weeks to 6 months

Question 22

Who was most afflicted by variant CJD

Answer 22

Adolescents (occasional cases in adults over 50)

Question 23

What is the clinical presentation/duration of vCJD

Answer 23

Psychiatric disturbance and complaints of sensory symptoms like limb pain
Duration 14 months

Question 24

3 regulations to control BSE

Answer 24

1. Feed bans
2. Specified risk materials bans (removed brain and spinal cord and destroy)
3. Regulation of rendering (autoclave slaughter materials)