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PrionPrusiner prion
(TSE)
(BSE) scrapie)(CJD)
GSS prion
Prion
Prion PrPc PrPsc PrPsc
PrPsc PrPc PrPsc
PrP PrPc Prion

TSE
1.
()
2. (autosomal)(dominant)
()
PrPsc PrPc
proteaseK
amyloid

prion (ataxia)
(
) 50%

spongiform encephalophathies
PrPsc

(astrocytogliosis)
PrP PrP

Disease description
prion disease
prion protein, PrP
spongiform
encephalopathysporadic, s-CJD
8090

Bovine Spongiform Encephalopathy, BSE


variant CJD, v-CJD 14 80
35 99.0
dementia

70EEG95 14-3-3
14-3-3
3 12
4 7
10 20
1/3
GSS Gerstmann-Straussler-Scheinker syndrome


Alzheimer disease
2005 17

1996 variat CJD, vCJD

29 1573
14 7
90

14-3-3