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Block Two Cell Bio > DO NOT STUDY Transmissible Spongiform Encephalopathies, Clinical > Flashcards

DO NOT STUDY Transmissible Spongiform Encephalopathies, Clinical Flashcards

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1
Q

Features Transmissible spongiform encephalopathies

A

non febrile, slowly degenerative, transmissible, fatal diseases affects CNS; associated with aberrant prior protein expression, spongiform brain lesions, no immune response to infective agent; associated with aberrant prion protein expression, not viral

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2
Q

Histology of transmissible spongiform encephalopathies

A

clear vacuoles in neuron

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3
Q

Etiology TSE

A

prion protein gene encoded by host and its expression is not indicative of a specific disease; aberrant conformation denotes dx; protein synthesized in ER eventually inserted into PM

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4
Q

physiological function TSE

A
  • cytoprotective (protect from oxidative stress)
  • binds copper
  • signaling molecule
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5
Q

infectious prion

A

aberrantly folded PrP = PrPsc is infectious prion resembles normal so can still insert into PM leads to aggregation and eventually neurodegenteratoin

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6
Q

normal prion formation

A

produced in ER -> Golgi -> PM

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7
Q

TSE classification requirements

A
  1. Prolonged incubation period
  2. slowly degenerative CNS signs
  3. Spongiform brain lesions
  4. transmissible to mice
  5. Presence of abnormal prion protein (PrPsc)
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8
Q

Human TSEs

A
  • Kuru (specific nerve degeneration, found in papa New Guinea)
  • creuzfeldtz-jacob disease (sporadic, familial, iatrogenic subtypes)
  • New variant creuzfeldtz-jacob disease (human form BSE)
  • Gerstmann-Straussler-Scheinker syndrome
  • Fatal familial insomnia
  • Multisystem atrophy
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9
Q

PrPc

A

host encoded, cellular membrane associated protein (this is normal one)

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10
Q

PrPsc

A

conformationally altered form of protein

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11
Q

PrPres

A

diagnostic technique with western blotting to detect PrPsc

Res= protease and hydrolysis resistant core of protein

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12
Q

PrPd

A

diagnostic approaches that don’t involve protein digestion such as immunohistochemistry (D= diseased prion protein)

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13
Q

Identifying pathogenic phenotype

A

western blot (reveal characteristics patterns of PrPres w/ variable molecular mass) or immunohistochemistry (detection PrPd in formalin fixed tissue)

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14
Q

Animal TSEs

A
  • Scrapies (sheep and goat)
  • Transmissible milk enceophalopathy
  • Chronic wasting disease (Cervids)
  • Bovine spongiform encephalopathy (BSE) (cows usually or cats, primates, captive non domestic ruminants, one goat)
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15
Q

TSE diagnostic tests

A 
Histopathology 
Presence of PrPsc:
-western blot
- immunohistochemistry
- ELISA
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16
Q

BSE

A 
bovine spongiform encephalopathy
-"Mad Cow Disease" don't call it that
-Host species: cattle, exotic ruminants, felines, humans, primates
-Animals get thin, splay legged 
NOT transmitted in milk
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17
Q

BSE clinical signs

A
  • long incubation period 4-7yrs
  • behavioral changes
  • motor incoordination
  • difficulty rising
  • weight loss with appetite
  • down or dead
  • may be carrying it but not showing signs -> it entering food chain
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18
Q

BSE susceptibility

A

low contraction rate but if you have it it is fatal; carriers have LONG incubation period; breed and genetic suseptablity not established yet but possible role

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19
Q

BSE transmission

A

non-contagious but infectious

  • oral transmission from ingestion of BSE-contaminated protein supplements; takes v little BSE brain material to contaminate
  • BSE instances decreased once ruminant to ruminant feeding stopped
20
Q

BSE UK Epidemic

A

recognized in 1986 ruminant to ruminant feed ban in 1988 takes a while to drop off bc long incubation, see more in active surveillance bc testing brains of everything but now see decline there too.. no new cases in last few years

21
Q

Where did a lot of BSE infected protein come from

A

UK

22
Q

Atypical BSE

A

These aren’t the ones from UK infected stuff, they are L type and H type; test for these with western blot; A typical has almost no spongiform changes while classical does in pons, obex, and midbrain

23
Q

Products free of PrPsc

A

Milk, muscle tissue, and blood

24
Q

Specified risk materials

A

BSE uptake in distal ileum by peyers patches; migrates to CNS where you get localization in the brain see in retinal neurons, retinal ganglia, and trigeminal ganglia; can affect spinal cord and DRG all these things are specialized risk materials which you keep out of the food chain

25
Q

Response to BSE

A

downer cows not allowed for human consumption; hold rendered carcas until BSE testing done; ban high risk material from animal feed; prohibit materials from animals >30 months entering food chain; ban air injection stunning; ban high risk material in animal feed;

26
Q

surveilance

A

-USDA implemented enhanced surveillance, test cattle of any age with CNS signs, BSE related clinical signs, or cow that died of unknown causes

27
Q

CJD resistant mice

A

from mutation from Force people in papa New Guinea, display resistance to CJD bc express novel human prion protein

28
Q

Scrapie

A

Sheep and goats; world wide except Australia and New Zeland; no known human health risk; first diagnosed in 1940s but was around wayyy before then; genetic / breed component

29
Q

scrapie clincal signs

A

subtile behavioral and temperamental changes, scratching and rubbing against fixed objects may lead to excoriation, loss of coordination, weight loss despite good appetite, biting of limbs, lip smacking, blindness, death (1-6 month clinical duration; death rapid after development of severe weakness)

30
Q

Scrapie transmission

A

Maternal through birth fluids, directly through blood, environmental transmission plays lesser role, 2-5 year incubation

31
Q

Scrapie breed susceptibility

A

black face sheep more susceptible (no cases in 2018 thus far)

32
Q

scrapie genetic susceptiblity

A

sheep prion protein 256 AA; diversity of genotype influences susceptibility to scrapie transmission; if you change certain AAs at certain positions can become more or less susceptable

33
Q

sheep and goat BSE susceptability

A

can give it to them if inoculate cortex BUT this doesn’t reflect nature so it is not likely to be transmitted; once case in France of goat picking this up naturally so contingency plans in place

34
Q

scrapie erradication

A

may be eradicated in our lifetime bc of scrapie eradication program; USDA monitoring closely surveillance and culling; encouraging submission goats and sheep over 18 months to diagnostic lab for necropsy and sample collection

35
Q

scrapie testing

A

western blot with scrapie specific MoAb reacts with true scrapie; in live sheep get lymphoid tissue from third eye lid or rectum; in dead sheep test opex (cd pt brain stem) and lymph node (usually retorpharyngeal) (test lymph bc lymph carries prion throughout body); confirmatory tests IHC, Western-blot, ELISA

36
Q

why test opex

A

highest number of nuclei so more likely to find something

37
Q

Chronic wasting dx

A

v common in mountain west of US; deer, elk, reindeer and moose affected by this (cervids); known since 1967 as TSE in 1978; age 17 months to >15yrs (avg 3-5 yrs); no strict seasonality; clinical duration days - 1yr but usually months; incubation period min 17 months max unknown

38
Q

Chronic wasting dx clinical signs

A

Ataxia and wide stance, subtle head tremors, periods of somnolence (head and ears down), ravenous appetite with weight loss, terminal polydipsia and polyuria, excessive salivation, found near water, may die of aspiration pneumonia

39
Q

Chronic wasting dx histology

A

lesions focused in int grey, bilateral sym, spongiform change; neuronal vacuolization with astrocyte hyperplasia is common;

40
Q

chronic wasting dx testing

A

detection PrPsc via IHC best way to visualize protein; nuclei of vagus usually most affected, protein can be found in CNS, PNS, lymphoid tissue; variety of ELISA and immunoblot tests

41
Q

chronic wasting dx location

A

common in US in central rockies, some in N Canada, also in Norway and S Korea (S Korea bc Canadian imports), about 20 cases so far in Scandinavia they’re V panicked bc could effect tourist industry bc reindeer (found in Finland); unknown how it got there, may represent less transmissible atypical form

42
Q

Chronic wasting dx transmission

A

more like scrapie than BSE; environmental contamination (spread in urine, feces, and saliva) agents persist in soil and other environmental areas, high prevalence in heavily contaminated areas (carcas decomposes near water easy spread and spreads to soil); agent spreads efficiently with in a herd, role of maternal transmission but appears to be of lesser importance

43
Q

CWD origin

A

origin is unknown maybe derivative from scrapie that affects cervids but it is currently unknown

44
Q

CWD transmission to rumminants

A

can be transmitted to cattle cerebrally but is atypicall of BSE but not all get it this way and no transmission from oral route or direct exposure;

45
Q

CWD genetic susceptiblity

A

AAs at certain positions in endogenous prion protein strongly associated with susceptibility to infection

46
Q

CWD public health significance

A

no evidence human transmission, most cases related to other human TSE conditions; appears there is considerable species barrier for TSE transmission to humans appears to be V specific to cervid populatoin

47
Q

CWD vaccine

A

CSU working on this; potential for vaccine response indicated thus far

Block Two Cell Bio (9 decks)

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