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Prions, prionoids and protein misfolding disorders

delete2018-04-30
delete214
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C
Claudia Scheckel
A
Adriano Aguzzi *
DOI:10.1038/s41576-018-0011-4delete
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Abstract

Abstract

En 中文
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 infectious. We now know that prions consist of PrPSc, the pathological aggregated form of the cellular prion protein PrPC. Over the years, the term has been semantically broadened to describe aggregates irrespective of their infectivity, and the prion concept is now being applied, perhaps overenthusiastically, to all neurodegenerative diseases that involve protein aggregation. Indeed, recent studies suggest that prion diseases (PrDs) and protein misfolding disorders (PMDs) share some common disease mechanisms, which could have implications for potential treatments. Nevertheless, the transmissibility of bona fide prions is unique, and PrDs should be considered as distinct from other PMDs.
Keywords:
CREUTZFELDT-JAKOB-DISEASE
GLUTAMATE-RECEPTOR 5
ENDOPLASMIC-RETICULUM STRESS
FATAL FAMILIAL INSOMNIA
GENETIC RISK-FACTORS
A-BETA
ALZHEIMERS-DISEASE
MOUSE MODEL
MUTANT P53
COGNITIVE IMPAIRMENT
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Key information extracted from the uploaded paper, including a brief overview, abstract, background, key highlights, visual analysis, and future outlook.

Journal

Nature Reviews Genetics cover
Nature Reviews Genetics
IF:
52
Papers:
4.0K
Citations:
4.3W

Organization

U
university of zurich
Scholars:
5.0W
Papers: 3.9W
Citations: 65