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Health Science > Prion Diseases > Flashcards

Prion Diseases Flashcards

Caoimhe

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1
Q

What are abnormal prions

A

Abnormal prions are infectious misfolded proteins that can trigger abnormal folding of other prions

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2
Q

Which level of protein structure do prions affect

A

Secondary structure

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3
Q

What is the largest class of protein secondary structures

A

alpha helices

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4
Q

Function of alpha helices

A

They have a major role in mediating protein-protein interactions (allows hydrogen bonding)

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5
Q

What does CJD stand for

A

Creutzfeldt-Jakob disease

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6
Q

what does vCJD stand for

A

Variable Creutzfeldt-Jakob Disease

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7
Q

What does BSE stand for

A

Bovine Spongiform Encephalopathy

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8
Q

Description of CJD

A

A transmissible subacute spongiform encephalopathy

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9
Q

Subacute meaning

A

There is a very long incubation time (long time between getting the disease and showing symptoms)

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10
Q

What does encephalopathy mean

A

Brain disease

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11
Q

What does spongiform mean

A

Brain tissue turns into a sponge like pattern

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12
Q

CJD aetiology (3)

A

1.Sporadic -> (majority of cases), hypothesised to occur as a result of aging cellular machinery, sporadic mutation of prion protein gene PRNP
2.Familial -> less than 10% of CJD is inherited in an autosomal dominant manner, mutation of prion protein gene also
3.Acquired -> all types are transmissible

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13
Q

What occurs to result in the mutation of the prion protein gene (PRNP)

A

Substitution of one amino acid to another at position 129 of PRNP (prion protein gene)

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14
Q

3 ways CJD can be acquired

A
  1. Transmission via exposure to brain / spinal tissue from infected person
  2. Canabalism -> Kuru, a prion disease, among Fore people in Papua New Guinea due to funerary canabalism
  3. Routine sterilisation procedures may fail to inactivate infectious prions
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15
Q

5 types of prion disease

A
  1. CJD
  2. vCJD
  3. Kuru
  4. Scrapie -> sheep
  5. BSE/mad cow disease -> cows
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16
Q

worldwide incidence of CJD

A

1 to 2 cases per one million people per year

17
Q

Why is the incidence of CJD increasing (5)

A
  1. Increased surveillance
  2. Aging population
  3. Increasing population
  4. Clinical awareness
  5. Diagnostic methods
18
Q

What is vCJD

A

The human equivalent of mad cow disease

19
Q

Aetiology of vCJD (3)

A
  1. Transmission via eating BSE infected beef
  2. Genetic susceptibility
  3. Blood product transmission possible
20
Q

When was the BSE epidemic in Ireland

A

1996

21
Q

When did the no. of BSE cases in Ireland peak and how many cases were there

A

The cases peaked in 2002 with 333 cases

22
Q

PATHOGENESISSSS

When does prion disease occur

A
  • When normal prion proteins (found on the surface of many cells) become abnormally folded.
  • These trigger abnormal folding of other prion proteins causing clumps and consequent brain damage.
23
Q

PATHOGENESISSSS

What is abnormal folding in terms of protein structure

A

Alpha helices to beta sheets

24
Q

PATHOGENESISSSS

What does PrPc mean and where is it found

A

PrPc = normal prion protein, a normal consitute of brain tissue in all mammals

25
Q

PATHOGENESISSSS

Describe PrPp (infectious prion)

A

PrPp (infectious prion) is found in infectious material, has a different structure and is resistant to proteases

26
Q

PATHOGENESISSSS

What conversion does PrPp (infectious prion) and PrPsc (infectious prion, named after scrapie in sheep) show

A

The conversion of alpha helices to beta sheets (abnormal folding)

27
Q

PATHOGENESISSSS

What way are the amino acids folded in PrPc (normal prion protein)

A

PrPc has amino acids in alpha helix with less than 5% of beta sheets

28
Q

PATHOGENESISSSS

What way are the amino acids folded in PrPp (infectious prion protein)

A

Majority of alpha helices are converted into beta sheets resulting in more than 50% of beta sheets

29
Q

Signs and Symptoms: Early Stage (4)

A
  1. Memory problems
  2. Behaviourial changes
  3. Poor coordination
  4. Visual disturbances
30
Q

Signs and Symptoms: Late Stage (9)

A
  1. Dementia
  2. Involuntary movements
  3. Blindness
  4. Coma
  5. Psychosis
  6. Anxiety
  7. Depression
  8. Paranoia
  9. Obsessive compulsive symptoms
31
Q

CJD tx

A

Symptom Management only:
1. Opioids for pain relief
2. Anticonvulsants/sedatives/skeletal muscle relaxants for involuntary movements
3. Antidepressants and sedatives for anxiety and depression
4. Palliative care

32
Q

CJD prognosis

A
  • Fatal
  • 70% die within a year of diagnosis
33
Q

CJD diagnosis (6)

A
  1. Signs & symptoms
  2. Brain MRI
  3. Elevated tumour marker neuron-specific enolase
  4. Elevated 14-3-3 protein on CSF analysis
  5. Genetic testing
  6. Brain biopsy to confirm -> post mortem

Health Science (10 decks)

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