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COM MOD 2.2 Prions > Prions > Flashcards

Prions Flashcards

1
Q

What are the 4 Human Prion Diseases?

A

CJD- Creutzfeld-Jakob Disease
GSS- Gerstmann-Strauddier Scheinker Disease
FFI- Fatal Familial Disease
Kuru

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2
Q

What are the 4 kinds of CJD?

A

sporadic
new variant
familial
iatrogenic

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3
Q

How does Kuru occur?

A

ritualistic cannibalism for ritualistic mourning of the dead

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4
Q

What are the common characteristics of the Prion diseases?

A 
long incubation time
gradual increase in severity
no host immune response
non-inflammatory process in brain
neruopathological findings
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5
Q

What are the neuropathological findings in prion diseases?

A

macroscopic exam is normal
microscopic spongiform changes- small empty vacuoles, neuronal loss, amyloid plaques, and accumulation of prion protein (PrP)

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6
Q

What are the neurological symptoms in prion diseases?

A 
Rapid progressive dementia
Psychiatric symptoms
Cerebellar symptoms (ataxia)
Involuntary movements (myoclonus)
Fatality
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7
Q

What is the major component of Prions?

A

prion protein (PrP)

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8
Q

What is PrPc?

A

Cellular isoform of PrP

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9
Q

What is PrPsc?

A

disease associated isoform of PrP–> SCrapie

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10
Q

What is the difference between PrPc and PrPsc?

A

the conformation

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11
Q

What are the 3 etiologies of PrP?

A

Inherited- mutations in PrP gene cause spontaneous PrPsc formation
Infectious- exogenous PrPsc causes PrPc to turn into PrPsc
Sporadic- unknown mechanism

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12
Q

What is the transmission process of Prions?

A

inoculation of oral

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13
Q

What is the golden standard for diagnosing Prion diseases?

A

histopathological exam and immunostaining for PrPsc in brain tissue

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14
Q

What are other findings in Prion disease diagnosis?

A

tau plaques, hockey stick looking MRI, tonsil biopsy for vCJD

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15
Q

What are the characteristics of Kuru?

A

infectious
ataxia, myoclonus, dementia
months before death

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16
Q

What are the characteristics of vCJD?

A

infectious
ataxia, dementia, psychiatric changes
month to years before death

17
Q

What are the characteristics of FFI?

A

inherited
sleep disturbances and dementia
1 year before death

18
Q

What are the characteristics of GSS?

A

inherited
ataxia and dementia
a few years before death

19
Q

What is the etiology of sCJD?

20
Q

What is the etiology of fCJD?

21
Q

What is the etiology of iCJD?

A

infectious

22
Q

What are the symptoms of sCJD, fCJD, and iCJD?

A

ataxia, myoclonus, and dementia

23
Q

What is the duration before death of sCJD, fCJD, and iCJD?

A

1 to a few years before death

24
Q

What are the infectious prion diseases?

A

kuru, vCJD and iCJD

25
Q

What are the inherited prion disease?

A

FFI, GSS, and fCJD

COM MOD 2.2 Prions (1 decks)

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