Prion—1997 Nobel Prize

Prion1997 Nobel Prize

CGU MIRS99

Stanley B. Prusiner prion 1997

prion

1.Kuru2.CJD3.GSS(Gerstmann-Strussler-Scheinker disease)4.FFI(fatal familiar insomnia)

Prion Proteinaceous infectious particlesGPIPrP PRNP gene

PRNPName of PRNPPrion protein (p27-30)Normal function making Prp , transport ionsWhere is the gene locatedRelated conditionsMutation PRNP ,polymorphism

PrPcPrion protein cellular Glycoprotein reside at nerve cellProtease K (protein-digesting enzyme)Prp-sen

PrPscPrion protein scrapiePrPc,Different in secondary structure (same primary structure)Protease kunstable

PrPcPrPsc2Refolding:PrPscPrPc refoldingPrPsc,heterodimerhomodimer,PrPcSeeding:PrPscoligomer,PrPsc,PrPc

prion(PrPsc)PrPcPrPc,(GroEl)

Heat shock protein:1.2.3. 4.

Prion

:kuru,BSE(Bovine Spongiform Encephalopathy), CJD

-

PrPsc,(amyloid),

(1)prionprion

(2)chlorpromazinev()prion

Prion,,,PrionPrion,!

NCBI PubMedhttp://brc.se.fju.edu.tw/nobelist/199x/p1997.htm Genetic home referencePrp structure