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15 Cards in this Set
- Front
- Back
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definition of a prion
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infectious agent that consists only of a protein; long incubation period and can not be destroyed; no immune response
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PrP protein
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only prion protein; misfolding (diff 3d shape) causes disease
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PrPc vs PrPsc (4)
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PrPsc is Beta-sheets
PrPsc forms amyloid fibrils PrPsc is resistant to degradation PrPsc converts PrPc to PrPsc |
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PrPsc can arise in 3 ways
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1) infection - PrPsc introduced to body
2) familial - AA changes lead to spontaneously folding 3) sporadic - randomly folds on its own |
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prion disease characteristics
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different confromations of PrPsc; long incubation times, fatal, damaged neurons appear vacuolated
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most prevalent prion disease, bariable presentation, progresses rapidly, prominent cognitive decline and myoclonus; 68 y/o median
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sporadic CJD
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Dx of CJD
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clinical manifestation, supported by 14-3-3 protein in CSF, confirmed by PrPsc in tonsils or brain
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infectious prion disease in New Guinea due to cannibalism
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Kuru
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ingestion of infected beef from england; median age of 28 y/o; pulvinar sign on MRI
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vCJD
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bovine spongiform encephalopathy
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late 80s, early 90s; epidemic of vCJD
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Scrapie
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endemic in sheep; not transmissible to humans
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Chronic wasting disease
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deer and elk; not transmissible to humans
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CJD resulting from medical procedure linked to contaminated HgH and surgical instruments
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Iatrogenic CJD
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Inherited prion disorders
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Gerstmann-Strausler-Scheinker (GSS) and Fatal Familial Insomnia (FFI); both autosomal dominant with high penetrance; AA changes in PrP
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Differences between CJD and GSS
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GSS occurs in 20s-40s with a disease duration of 7 years
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