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Animal Health and Disease & Animal and Vet Genetics > ANI SCI 320 Lecture 11: Prions > Flashcards

ANI SCI 320 Lecture 11: Prions Flashcards

EXAM 2

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1
Q

What are the 4 prion diseases in humans?

A
  1. Kuru
  2. Creutzfeldt Jakob Disease (CJD)
  3. Fatal Familial Insomnia (FFI)
  4. Gerstmann Straussler Syndrom (GSS)
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2
Q

What is a prion disease in cattle?

A

Bovine Spongiform Encephalopathy (BSE) (“Mad Cow Disease”)

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3
Q

What is a prion disease in sheep/goats?

A

Scrapie

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4
Q

What is a prion disease in deer/elk?

A

Chronic Wasting Disease (CDW)

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5
Q

What are TSEs?

A

Transmissible Spongiform Encephalopathies

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6
Q

What are 4 characteristics of TSEs?

A
  1. Spongiform (Brain has holes in it)
  2. Neuronal loss, gliosis and astocytosis
  3. Atrophy of brain tissue
  4. Accumulation of misfolded prion plaques (vacuoles and build up of plaque)
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7
Q

How do prions affect the cerebral cortex?

A

Loss of memory and mental ability, and sometimes visual impairment (CJD)

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8
Q

How do prions affect the thalamus?

A

Insomnia (FFI)

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9
Q

How do prions affect the cerebellum?

A

Problems with body coordination and movement and difficulties walking (kuru, GSS)

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10
Q

How do prions affect the brain stem?

A

Mad Cow Disease

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11
Q

What are the 5 characteristics of a prion infection?

A
  1. Encephalitis (Inflammation of the brain)
  2. Widespread of neuronal loss
  3. Loss of motor control
  4. Dementia
  5. Paralysis
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12
Q

Where was kuru identified?

A

Papua New Guinea by Robert and Louise Glasse in 1950

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13
Q

What was an interesting trend of the kuru disease?

A

1% fore tribe was afflicted
Mostly women, some children, and FEW adult males

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14
Q

What are the symptoms of kuru?

A

Headache, joint pain, difficultly walking, and death within 12 months

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15
Q

What was suggested to be associated with the kuru disease?

A

endocannibalism - people ate the brains of dead people as part of a funeral ritual (religious practice)

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16
Q

Who suppressed cannibalism among North Fore? Did this solve the kuru problems.

A

Australian government
Yes - no child sense has died of kuru

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17
Q

Who proved that its whats in the brain that has something to do with kuru disease? With what subjects?

A

Carlton Gadjusek
Used inoculated chimps with brain extracts of kuru victims and all chimps died after 50 months

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18
Q

TRUE or FALSE? No unique antibodies were associated with disease, no virus particles or aberrant nucleic acids were identified with Gadjuseks subjects?

A

TRUE

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19
Q

Kuru research gave rise to what need?

A

Animal models to study TSEs

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20
Q

What disease has many similarities to kuru in terms of symptomatology and eitology?

A

Scrapie Disease in sheep

21
Q

What was different about scrapie disease?

A

Could be transmitted to hamsters and mice while kuru could not.

22
Q

What was the first good animal model for TSE studies?

A

Mice infected with scrapie

23
Q

Why was using mice for scrapie studies better than sheep?

A

2 month incubation in rodents whereas sheep it would be 2-5 years before you can do research

24
Q

What did scrapie agent find?
What did this tell us about the disease?

A
  1. Nuclease resistant
  2. UV and heat resistant
  3. Sensitive to protease (ONLY at high levels) and protein denaturants
    TELLS US: its a protein based infection
25
Q

In terms of BSE what was in placed in the 1970s in Britain? Why did this lead to BSE?

A

Hydrocarbon solvent extraction of meat and bond meal for cattle feed was abandoned in Britain. (Feeding the same components of an animal back to its species)

Removal of hydrocarbon solvent no longer killed infectious agent therefore BSE emerged

26
Q

In terms of BSE what occurred in 1986-87?

A

BSE emerged (~700 BSE infected cows)

27
Q

In terms of BSE what occurred in 1988?

A

~7,000 infected cows. BSE became a “reportable” disease. Epidemiology suggested a prion disease and MBM use was abandoned

28
Q

What is the incubation period for BSE?

A

5 Years

29
Q

In terms of BSE what occurred in 1989?

A

Human consumption of bovine CNS tissue banned based on fears of transmission to humans

30
Q

In terms of BSE what occurred in 1996?

A

A new type of CJD appeared in Britain and France (Young patients) and different neuropathology. Linked with consumption of BSE-contaminated beef

31
Q

Prions are short for……

A

Proteinaceous infectious particle

32
Q

TRUE or FALSE? TSEs are always fatal?

A

TRUE

33
Q

Is there a treatment for TSE?

A

No treatment that can halt progression of TSEs

34
Q

Normal prions are common where?

A

Brain cells

35
Q

What do normal prions contain?

A

200-250 amino acids twisted into 3 telephone chord like coils known as helices with tails of more amino acids

36
Q

How are mutated and infectious prions different from normal prions?

A

Build with the same amino acids but takes a different shape

37
Q

How small is a prion?

A

100 times smaller than the smallest known virus

38
Q

What are the 3 types of prion diseases?

A
  1. Sporadic
  2. Infectious
  3. Familial/Hereditary
39
Q

What are the 3 characteristics of sporadic TSEs?

A
  1. No prion protein mutation (Just misfolding)
  2. 1-2 million human infected world-wide (later in life)
  3. Cause is unknown
40
Q

What are examples of infectious TSEs?

A

Kuru, BSE, and Scrapie

41
Q

How are infectious TSEs spread?

A
  1. Consumption of infected material
  2. latrogenic spread (organ transplant)
  3. Blood Transfusion
42
Q

What are 2 characteristics of Familial/Hereditary TSEs?

A
  1. Due to autosomal dominant mutation of PrP
  2. Inherited - at least 10-15% of total human TSE cases
43
Q

What are the 5 properties of infectious prions?

A
  1. They are resistant to degradation by proteases (leads to accumulation)
  2. beta sheet structure has high affinity for other beta sheet structures (form obligometric insoluble aggregates that turn form toxic amyloid plaques) interferes with cell tissue function and death of cells/tissues
  3. Extremely resistant to heat and chemicals
  4. Difficult to decompose biologically and they survive in the soil for many years
  5. Prions are not nucleic acids
44
Q

How do infectious prions propagate?

A

During the oligomerization the prions corrupt the native form of the protein into a transmissible disease conformation

45
Q

What causes the familial mutation?

A

Mutation on the protein changes folding

46
Q

What causes the sporadic arise?

A

There is a misfolding of the protein that generally cannot be explained?

47
Q

What causes the acquired prion or heteronucleant?

A

Foreign prion enters the body by consumption or contraction and changes the protein folding

48
Q

What causes a small molecule specific destabilizer that causes prions?

A

Molecule comes into and destabilizes the protein folding

Animal Health and Disease & Animal and Vet Genetics (18 decks)

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