Lecture 21: TSE

Question 1

T or f: chronic wasting disease is reportable

Answer 1

true

Question 2

how do you dx chronic wasting disease

Answer 2

postmortem ELISA, IHC of brainstem (obex) and retropharyngeal LN

Question 3

what would a positive IHC staining for CWD look like

Answer 3

spongiform degeneration in obex (brainstem) with CWD + staining in RLN +/- obex

Question 4

what is the difference between normal prion and infectious prion

Answer 4

protein misfolding
Normal prion: PrPc
Infectious prion: PrPres

Question 5

Infectious prions are very resistant to __ and __

Answer 5

disinfection and sterilization

Question 6

long incubation periods for prion disease but still ultimately___

Answer 6

fatal

Question 7

t or f: prion diseases have no specific immune response

Answer 7

true

Question 8

t or f: prion disease is zoonotic

Answer 8

true

Question 9

what is the cellular prion protein all vertebrates have

Answer 9

PrPc

Question 10

cellular prion protein is a ___anchored extracellular monomer

Answer 10

membrane anchored

Question 11

what is cellular prion protein degraded by

Answer 11

ubiquitin-proteasome system

Question 12

infectious prion protein sequence is identical to cellular protein but differences in __

Answer 12

tertiary structure (beta sheets form)

Question 13

infectious prion proteins are extremely resistant to __

Answer 13

harsh physical treatments

Question 14

infectious prion proteins form __ that obstruct __

Answer 14

fibrillar amyloid that obstruct UPS

Question 15

What diseases in humans are prion diseases similar too

Answer 15

Alzheimer’s and Parkinson’s

Question 16

how is scrapie and CWD spread

Answer 16

biological fluids, environmental contamination

Question 17

True or false scrapie and CWD is zoonotic

Answer 17

false

Question 18

how do cows get BSE

Answer 18

meat and bone meal- consumption of infected tissues

Question 19

T or f: BSE is zoonotic

Answer 19

true

Question 20

what Are dead end hosts for BSE

Answer 20

humans and cattle

Question 21

what pathogenesis for TSE

Answer 21

1. Oral exposure
2. Centripetal propagation (Periphery to CNS or lymphatics)
3. Centrifugal dissemination (muscle, oral and renal tissues, antler, caudal GI tract, nasal epithelium)

Question 22

what TSE’s travel on PNS to. CNS

Answer 22

CWD, scrapie, BSE, CJD

Question 23

what TSE’s can travel on lymphatics to CNS

Answer 23

CWD and scrapie

Question 24

Which TSE’s do centrifugal dissemenation

Answer 24

CWD and scrapie

Question 25

what is incubation period and susceptibility for prion disease dependent on

Answer 25

agent, host (genotype), route of inoculation, dose

Question 26

How does the host genotype alter susceptibility to prion disease

Answer 26

subtle alterations in host prion compared to infectious PrP can make it so can’t infect or incubation period much longer

Question 27

what is the host genotype in sheep for prion disease that is resistant

Answer 27

ARR

Question 28

in white tailed deer, mule deer, and elk various __affect susceptibility from prion disease, but not are 100% protective

Answer 28

various polymorphisms

Question 29

what is species barrier and how does that relate to prion disease resistance

Answer 29

Not all species have exact same prion and therefore not able to be infected by all infectious prion diseases

Question 30

what species are affected by CWD and who is not

Answer 30

cervids, lab animals, not humans

Question 31

What species is affected by BSE

Answer 31

cattle, several domestic and wild species, as well as humans

Question 32

what are TSE clinical signs

Answer 32

downer cattle, incoordination, persistent scratching, hypersalivation, PU/PD

Question 33

What samples do you collect for TSE dx: BSE and CWD

Answer 33

BSE: obex CWD: RLN and obex

Question 34

how do you dx TSE and what are you looking for

Answer 34

IHC: gold standard ELISA Spongiform degeneration

Question 35

what is control for scrapie

Answer 35

depopulation Selective breeding (ARQ/ARR)

Question 36

what is control of CWD

Answer 36

depopulation

Question 37

what is control for BSE

Answer 37

MBM (meat, bone meal) bans, depopulation

Question 38

what is pathogenesis for BSE

Answer 38

PNS—> CNS

Question 39

how is CWD transmitted

Answer 39

Horizontal (excrete/environment)

Question 40

how is BSE transmitted

Answer 40

iatrogenic (MBM)