Failure of elimination of proteins from the brain is a major feature in many
neurodegenerative diseases. Insoluble proteins accumulate in brain parenchyma and in …

Prion diseases are a group of infectious neurodegenerative diseases with an entirely novel
mechanism of transmission, involving a protein-only infectious agent that propagates the …

Within the extensive range of self-propagating pathologic protein aggregates of mammals,
prions are the most clearly infectious (eg,∼ 10 9 lethal doses per milligram). The structures …

Little is known about the structural basis of prion strains. Here we provide a high (3.0 Å)
resolution cryo-electron microscopy-based structure of infectious brain-derived fibrils of the …

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 …

The structure of the infectious prion protein (PrPSc), which is responsible for Creutzfeldt-
Jakob disease in humans and bovine spongiform encephalopathy, has escaped all attempts …

The structures of prion protein (PrP)–based mammalian prions have long been elusive.
However, cryo-EM has begun to reveal the near-atomic resolution structures of fully …

Proteinopathies represent a group of diseases characterized by the unregulated misfolding
and aggregation of proteins. Accumulation of misfolded protein in the central nervous …

A variety of physiological functions, not only restricted to the nervous system, are discussed
for the cellular prion protein (PrP C). A prominent, non-physiological property of PrPC is the …

Proteolytic processing of the cellular and disease-associated form of the prion protein leads
to generation of bioactive soluble prion protein fragments and modifies the structure and …