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Nihon Rinsho. 1993 Sep;51(9):2494-502.

[A trend of molecular genetics on prion diseases and prion protein].

[Article in Japanese]

Author information

  • 1Department of Veterinary Public Health, School of Veterinary Medicine, Obihiro University of Agriculture and Veterinary Medicine.

Abstract

Infectious amyloid filaments designated as prion rods or scrapie associated fibrils (SAF) present in brain tissues affected by transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS) and kuru of humans, and scrapie of sheep. A hydrophobic glycoprotein, PrPSc is a major component of SAF, and is known to be associated with the infectivity of these diseases. Both PrPSc and the normal isoform of this glycoprotein, PrPC are encoded by a single host gene, PrP gene, and the conversion of PrPC to PrPSc is a posttranslational event. Several mutations on the PrP gene are associated with variations of the phenotype and the occurrence in familial CJD and GSS.

PMID:
8411733
[PubMed - indexed for MEDLINE]
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