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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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Updated: Jun 20, 2025

Prion Safety Laboratory Swipe Test
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Prion diseases, always a threat?

Yuji Saitoh1, Hidehiro Mizusawa2

  • 1Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo 183-0042, Japan.

Journal of the Neurological Sciences
|July 19, 2024
PubMed
Summary

Prion diseases, caused by infectious proteins, are serious neurodegenerative disorders. Emerging evidence suggests other neurodegenerative diseases may share prion-like transmission, posing a growing threat.

Keywords:
Creutzfeldt-Jakob diseaseEnvironmentally acquired human prion diseasesIatrogenic Alzheimer's diseaseIatrogenic Creutzfeldt-Jakob diseasePrionVariant Creutzfeldt-Jakob disease

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Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Protein Misfolding Diseases

Background:

  • Prion diseases, like Creutzfeldt-Jakob disease (CJD), are fatal neurodegenerative disorders caused by proteinaceous infectious particles (prions).
  • These diseases affect humans and animals, with forms including sporadic, genetic, and acquired (e.g., iatrogenic CJD, variant CJD).
  • While some prion diseases are controlled, others like chronic wasting disease are spreading.

Purpose of the Study:

  • To highlight the threat of prion diseases and their potential link to other neurodegenerative conditions.
  • To emphasize the need for strategies to combat prion diseases and understand their broader implications.

Main Methods:

  • Review of existing literature on prion diseases and transmissible spongiform encephalopathies.
  • Analysis of recent findings on protein transmission in neurodegenerative diseases like Alzheimer's and Parkinson's.

Main Results:

  • Prion diseases are characterized by protein misfolding and transmission, affecting diverse species.
  • Proteins implicated in Alzheimer's and Parkinson's disease exhibit prion-like transmission characteristics.
  • Amyloid-beta transmission is suggested in certain iatrogenic CJD and cerebral amyloid angiopathy cases.

Conclusions:

  • Prion diseases, exemplified by CJD, are a significant public health concern, especially in aging populations.
  • The prion concept is crucial for understanding various neurodegenerative diseases.
  • Developing strategies against prion diseases is vital for advancing therapies for a range of neurological disorders.