Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and
bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann …

PrPc is a host protein anchored to the outer surface of neurons and to a lesser extent of
lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is …

The normal cellular form of prion protein (PrPC) is a precursor to the pathogenic protease-
resistant forms (PrPSc) believed to cause scrapie, bovine spongiform encephalopathy (BSE) …

Background. We previously described two members of a family affected by an apparently
genetically determined fatal disease characterized clinically by progressive insomnia …

GERSTMANN–Sträussler syndrome is a rare familial neuro-degenerative condition that is
vertically transmitted, in an apparently autosomal dominant way1. It can also be horizontally …

Fatal familial insomnia (FFI) and a subtype of familial Creutzfeldt-Jakob disease (CJD), two
clinically and pathologically distinct diseases, are linked to the same mutation at codon 178 …

The cellular form of the prion protein (PrP c) is a glycoprotein anchored to the cell membrane
by a glycosylphosphatidylinositol moiety. An aberrant form of PrP c that is partially resistant …

Prion diseases are transmissible, progressive and invariably fatal neurodegenerative
conditions associated with misfolding and aggregation of a host-encoded cellular prion …

Prion protein (PrP) plays a crucial role in prion disease, but its physiological function
remains unclear Mice with gene deletions restricted to the coding region of PrP have only …