Prions are a type of intrinsically disordered protein that continuously changes conformation unless bound to a specific partner, such as another protein. Once a prion binds to another in the same conformation, it stabilizes and can form a fibril, leading to …

Here we report a near-atomic core structure of a brain-derived, fully infectious prion (263K strain). Cryo-electron microscopy showed amyloid fibrils assembled with parallel in-register intermolecular β sheets. Each monomer provides one rung of the ordered fibril core, with N-linked glycans and glycolipid anchors projecting outward.

1999年10月21日 · "'Prion' is a term first used to describe the mysterious infectious agent responsible for several neurodegenerative diseases found in mammals, including Creutzfeldt-Jakob disease (CJD) in humans.

2024年11月22日 · Prions are proteinaceous infectious particles implicated in fatal neurodegenerative disorders known as prion diseases. Herein, we provide an overview of prion biology, emphasizing the structural, functional, and evolutionary aspects of prions, along with their potential applications in protein engin …

2023年1月16日 · Transmissible spongiform encephalopathies (TSEs) or prion diseases are invariably fatal neurodegenerative disorders affecting mammals and include...

2013年11月5日 · Prion diseases are fatal, transmissible, neurodegenerative conditions of sporadic, infectious, iatrogenic or genetic origin. The infectious agent is termed a prion and consists of or coincides...

2019年11月30日 · Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are chronically neurodegenerative and potentially zoonotic pathological processes. 1 The prototypic prion disease was first detected in sheep called “ Scrapie ” whose pathogen was called scrapie PrP (PrP Sc). 2 Human TSEs include Creutzfeldt-Jakob disease (CJD ...

Prions are self-replicating protein aggregates and are the primary causative factor in a number of neurological diseases in mammals. The prion protein (PrP) undergoes a conformational transformation leading to aggregation into an infectious cellular ...

Coloured transmission electron micrograph (TEM) of scrapie-associated protein strands taken from the brain of an infected hamster. Scrapie is a transmissible spongiform encephalopathy (TSE), which causes brain degeneration in some animals.

Príon é uma proteína infecciosa capaz de provocar doenças cerebrais degenerativas que acometem diferentes espécies, inclusive os seres humanos. Os príons são o resultado de …