Prion diseases have stimulated intense scientific scrutiny since it was proposed that the
infectious agent was devoid of nucleic acid. Despite this finding, genetics has played a key …

The prion diseases are a family of rare neurodegenerative disorders that result from the
accumulation of a misfolded isoform of the prion protein (PrP), a normal constituent of the …

Genetic prion diseases (gPrDs) caused by mutations in the prion protein gene (PRNP) have
been classified as genetic Creutzfeldt–Jakob disease, Gerstmann–Sträussler–Scheinker …

▪ Abstract Prions are unprecedented infectious pathogens that cause a group of invariably
fatal, neurodegenerative diseases by an entirely novel mechanism. Prion diseases may …

Although prion diseases are generally thought to present as rapidly progressive dementias
with survival of only a few months, the phenotypic spectrum for genetic prion diseases …

Human prion diseases are a group of rare neurodegenerative disorders characterized by
the conversion of the constitutively expressed prion protein, PrP C, into an abnormally …

The N terminus of the scrapie isoform of prion protein (PrPSc) can be truncated without loss
of scrapie infectivity and, correspondingly, the truncation of the N terminus of the cellular …

Prion diseases in humans are neurodegenerative diseases which are caused by an
accumulation of abnormal, misfolded cellular prion protein known as scrapie prion protein …

Prion diseases are a family of transmissible, progressive, and uniformly fatal
neurodegenerative disorders that affect humans and animals. Although cross-species …

By far the largest known kindred with an inherited prion disease caused by a prion protein
(PrP) octapeptide repeat insertion mutation originates from southeast England. This …