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Microbiology Unit 2 > Prions > Flashcards

Prions Flashcards

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1
Q

What is sporadic Creutzfeldt-Jacob Disease? How is it NOT transmitted?

A

sporadic - no known cause
NOT transmitted person-to-person by blood transfusion
NOT transmitted by meat contaminated with BSE

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2
Q

what are sCJD, fCJD, and iCJD?

A

sporadic Creutzfeldt-Jacob Disease - no known cause
familial Creutzfeldt-Jacob Disease = inherited genetic risk
iatrogenic Creutzfeldt-Jacob Disease = exposure during medical procedures

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3
Q

What is spongioform encephalopothy?

A

prion disease

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4
Q

How is sCJD different from Alzheimer’s?

A

sCJD has more rapid progression of memory loss

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5
Q

What are the two cardinal clinical manifestations of sCJD?

A

rapidly progressive mental deterioration

myoclonus - provoked by startle

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6
Q

What led to the protein-only hypothesis for prion diseases?

A
  • prion diseases not associated with virus particles, bacteria, fungi
  • infectivity not associated with nucleic acid; prions are resistant to UV radiation and nucleases
  • no immune response to infection
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7
Q

What do skeptics say against the protein-only hypothesis for prion diseases, which are characterized by protein misfolding, similar to to Alzheimer’s, Parkinson’s, and Huntington’s?

A

the other diseases diseases are not infectious, but prions are
misfolded protein is not enough to transmit disease

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8
Q

What do skeptics say about the fact that spongioform diseases come in distinct strains which differ in their incubation period, symptoms, and effects on different brain regions?

A

differences are due to mutations in nucleic acids

long incubation time and rapid onset resemble HIV

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9
Q

What is PrP^c?

A

Cellular form of Prion Protein - membrane glycoprotein

Misfolding causes encephalopathy

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10
Q

What does PrP^c become after misfolding? What does this lead to?

A

PrP^sc - Scrapie form of Prion Protein

Prion disease

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11
Q

How can PrP^sc arise?

A 
mutation
exogenous sources (meat, blood)
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12
Q

How were 4 types of human PrP^sc detected?

A

digestion with proteinase K yielded 3 PrP bands

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13
Q

What is the model for Prion Self Replication?

A

Seed (nucleus) recruits PrP^c (native conformers) and converts them to PrP^sc (prion conformers)
seed increases in size –> amyloid fibre
fragmentation –> liberates new ends to allow for amplification; allows dissemination of infectious material

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14
Q

What is the diagnostic test for all forms of CJD?

A

brain biopsy

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15
Q

What three things characterize sCJD?

A

accumulation of PrP^sc
neuronal loss without inflammation
spongioform change

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16
Q

What is sCJD treatment and prognosis?

A

no treatment
median disease lasts 5-6 months
death after one year

17
Q

What is a type 4 prion which represents bovine-to-human transmission of BSE?

A

variant Cruetzfeldt-Jakob disease (vCJD)

18
Q

In what body parts of cattle is vCJD not found?

A

muscle

milk

19
Q

How is vCJD different from sCJD in clinical presentation?

A

vCJD has slower onset of loss of brain function

20
Q

what is the difference between detection of 14-3-3 protein in cerebral spinal fluid for sCJD and vCJD?

A

sensitive marker for sCJD

not for vCJD

21
Q

Can an abnormal MRI result be used to distinguish sCJD from vCJD?

A

yes - both are abnormal, but distinct

22
Q

What is the difference between periodic synchronous sharp wave complexes (PSWCs) seen via EEG in vCJD versus sCJD?

A

sCJD had sharp wave complexes

vCJD has slow waves

23
Q

Which form of CJD appears as diffuse staining?

A

sCJD

24
Q

Which form of CJD stains as a round amyloid core surrounded by a ring of spongioform vacuoles?

A

vCJD

25
Q

How does the mean onset age for vCJD compare with that of sCJD?

A 
vCJD = 29
sCJD = 65t
26
Q

What is BSE?

A

Bovine spongioform encephalopathy

27
Q

What sparked the peak of the BSE epidemic?

A

ban on ruminant feed

28
Q

Which type of CJD can be transmitted from person-to-person by blood transfusion or ingestion of food contaminated with BSE?

A

vCJD

29
Q

Which type of CJD has a peripheral pathogenesis (in tonsils, thymus, adrenal glands, lymph nodes, terminal ileum appendix, rectum)?

A

vCJD

30
Q

Which sites of symptoms do sCJD and vCJD share?

A 
brain
eyes
spinal chord
muscle
spleen
31
Q

Why is secondary latent transmission of vCJD a potential concern?

A

still carried in UK population

average incubation period of 20-3- years

Microbiology Unit 2 (14 decks)

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