Analysis of prion strains by PrPSc profiling in sporadic Creutzfeldt-Jakob disease.

ABSTRACT:

Background

Prion diseases are a group of invariably fatal neurodegenerative disorders affecting humans and a wide range of mammals. An essential part of the infectious agent, termed the prion, is composed of an abnormal isoform (PrPSc) of a host-encoded normal cellular protein (PrPC). The conversion of PrPC to PrPSc is thought to play a crucial role in the development of prion diseases and leads to PrPSc deposition, mainly in the central nervous system. Sporadic Creutzfeldt-Jakob disease (sCJD), the most common form of human prion disease, presents with a marked clinical heterogeneity. This diversity is accompanied by a molecular signature which can be defined by histological, biochemical, and genetic means. The molecular classification of sCJD is an important tool to aid in the un

SUBMITTER: Schoch G 

PROVIDER: S-EPMC1316067 | biostudies-literature | 2006 Feb

REPOSITORIES: biostudies-literature