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BDS 2: FHHD > M10 > Flashcards

M10 Flashcards

1
Q

What are Prion diseases?

A

Related group of rare, fatal brain diseases that affect animals, including humans

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2
Q

What is are Prion diseases also known as?

A

Transmissible spongiform encephalopathies (TSE)

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3
Q

Give some examples of Prion diseases (5)

A
  • Mad cow disease in cattle
  • Creutzfeldt-Jakob disease (CJD) in humans
  • Kuru in humans
  • Scrapie in sheep
  • Chronic Wasting Disease in deer and elk
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4
Q

How do Prion diseases occur?

A
  • Characterised by proteins that adopt alternative shapes in the brain tissues
  • The prion form of the protein clumps together and accumulate in brain tissue causing damage to the brain
  • These are prion forms of the protein
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5
Q

What are prions?

A

Shape shifting proteins

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6
Q

What is PrPC?

Where is it found?

A
  • The normal prion protein is called PrPC

- It is a plasma membrane glycoprotein found at the cell surface

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7
Q

Describe the secondary structure of PrPC

A

Its secondary structure is dominated by alpha helixes

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8
Q

What is PrPSc?

How has its protein structure changed from PrPC?

A
  • The disease-producing protein is called PrPSc (for scrapes)
  • The same amino acid sequence as PrPC, but now folded with more beta-sheets
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9
Q

What happens when PrPSc comes into contact with PrPC?

A

It converts PrPC into more PrPSc

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10
Q

How are prion aggregates formed?

A

Converting PrPC into more PrPSc and these all sticking together

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11
Q

What are amyloid deposits?

Name a disease that involves amyloid deposits

A
  • The deposits of PrPSc in the brain are called amyloid

- Alzheimer’s disease

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12
Q

What are the roles of proteasomes in cells?

A

Responsible for degrading misfolded or aggregated proteins

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13
Q

Why may proteasomes not stop prions?

A
  • The build-up of amyloid deposits overwhelms the capacity of the proteasomes to do their job
  • This leads to cell death
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14
Q

Name some inherited prions diseases (3)

A
  • Creutzfeldt-Jakob Disease (CJD)
  • Gerstmann-Sträussler-Scheinker disease (GSS)
  • Fatal Familial Insomnia (FFI)
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15
Q

How can people get CJD without inheritance? (2)

A
  • Accidental exposure to material contaminated with CJD prions (e.g surgical instruments)
  • Corneal transplants have also inadvertently transmitted CJD
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16
Q

What is Gerstmann- Sträussler- Scheinker disease (GSS) caused by? (2)

A
  • Inheritance of a PRNP gene containing mutations

- Transgenic mice expressing the P102L gene develop the disease spontaneously

17
Q

What is Fatal Familial Insomnia (FFI) caused by? (2)

A

Inherited a PRNP gene with asparagine instead of aspartic acid encoded at position 178 (D178N)

18
Q

What are the 4 stages of Fatal Familial Insomnia (FFI)?

A
  • Stage 1: Insomnia, panic attacks, phobias
  • Stage 2: Hallucinations, panic attacks
  • Stage 3: Complete Inability to sleep, rapid weight
    loss, early menopause, impotence.
  • Stage 4: Dementia and Death
19
Q

Give some examples of infectious prion diseases (4)

A
  • Kuru
  • Scrapie
  • Variant Creutzfeldt-Jakob Disease (vCJD)
  • Miscellaneous Infectious Prion Diseases
20
Q

How can scrapies be transmitted? (2)

A
  • Animal to animal

- Injection of brain tissue

21
Q

How does kuru disease occur?

A

Eating brain tissue

22
Q

What are the 3 stages of Kuru disease?

A
  • 1st stage: Unsteadiness, clumsiness, eye tremors, slurred speech.
  • 2nd stage: not able to walk without help, severe tremors, muscle jerking, loss of coordination, laughter outbursts, slurred speech.
  • Terminal stage: unable to sit up, tremors, incontinence, difficulty swallowing and death.
23
Q

What is another name for mad cow disease?

A

Bovine Spongiform Encephalopathy (BSE)

24
Q

How did mad cow disease arise?

A

Its origin appears to have been cattle feed that contained brain tissue from sheep infected with scrapie

25
Q

Describe the genealogy involved in Variant Creutzfeldt-Jakob Disease (vCJD)?

A

All the patients are homozygous for the susceptibility polymorphism of methionine at position 129

26
Q

How was mad cow disease combatted?

A

Ban on the use of bovine offal in baby foods

27
Q

What are some clinical and pathological features of vCJD? (3)

A
  • An early age of onset or death
  • A prolonged duration of illness
  • A predominantly psychiatric presentation including anxiety, depression, withdrawal behavioural change which progresses
28
Q

What are some control measures for prion diseases (3)

A
  • Recipients of Blood transfusions or blood products cannot be blood donors.
  • Use of disposables in surgical procedures
  • Cell lines banked post 1980 in the UK are generally not
    used for the production of vaccines or biotherapeutics

BDS 2: FHHD (34 decks)

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