Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible
neurodegenerative disease associated with the accumulation of misfolded prion protein in …

The most definitive classification systems for dementia are based on the underlying
pathology which, in turn, is categorized largely according to the observed accumulation of …

Some individuals with a particular disease-causing mutation or genotype fail to express
most if not all features of the disease in question, a phenomenon that is known as 'reduced …

Apolipoprotein E is immunochemically localized to the senile plaques, vascular amyloid,
and neurofibrillary tangles of Alzheimer disease. In vitro, apolipoprotein E in cerebrospinal …

More than 100,000 genetic variants are reported to cause Mendelian disease in humans,
but the penetrance—the probability that a carrier of the purported disease-causing genotype …

Human prion diseases can be sporadic, inherited, or acquired by infection. Distinct clinical
and pathological characteristics separate sporadic diseases into three phenotypes …

The fundamental event in prion diseases seems to be a conformational change in cellular
prion protein (PrPC) whereby it is converted into the pathologic isoform PrPSc. In fatal …

Of the 20 amino acids, the precise function of methionine (Met) remains among the least well
understood. To establish a determining characteristic of methionine that fundamentally …

We sequenced the prion protein gene and studied the biochemical characteristics and the
intracerebral distribution of protease‐resistant prion protein with Western blot and …

The molecular basis of strain variation in scrapie diseases is unknown. The only identified
component of the agent is the posttranslationally modified host prion protein (PrPSc). The …