L39_Prions Flashcards
What is the most common prion disease in humans?
CJD
What type of CJD is the most common?
sporadic or sCJD
Can sCJD be transmitted person to person by blood transfusion, what about vCJD?
sCJD-NO
vCJD-YES
Is sCJD related to human mad cow disease?
no
What main part of the brain does sCJD infect?
Cerebrum
What are the two cardinal symptoms of sCJD?
RAPID progressive mental deterioration and myoclonus (muscle twitching), myoclonus may have later onset
What is the normal form of the prion protein and what is the pathogenic form, what is the difference in their structure? What gene encodes the normal one.
The normal protein PRPc is encoded by the Prnp gene and is alpha helical. The pathogenic protein PRPsc is Beta sheet and can arise from either a misfolded PrPc or an exogenous source
How many classes of PrPsc and how are they differentiated?
4, differentiated by western blot (ratio of 3 bands)
What are the two classes of CJD that are most focussed on, which one is sCJD and which is vCJD?
Class 1 is sCJD and class 4 is vCJD
What is the only way to diagnose CJD?
Brain biopsy
What are two other diagnostic findings that can support sCJD?
abnormal MRI and EEG
What are the biopsy findings of sCJD?
Accumulation of PRPsc
Neuronal loss without inflammation
Spongiform Change
Are there treatments for sCJD?
NO, you’re fucked (5-6mo - 1 yr)
What demographic tends to suffer the most form sCJD?
Elderly, 65 and above
vast majority of deaths in in whites
With regard to vCJD, do muscle and milk of a cow contain BSE?
No, but if they are contaminated with it, you can get it
