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Related Concept Videos

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Subviral Agents

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Subviral agents are infectious entities that resemble viruses but lack one or more viral components, such as a capsid or essential replication machinery. These agents include viroids, prions, and satellites, each possessing distinct structural and functional characteristics that influence their mode of infection and replication.Viroids are the simplest subviral agents, consisting of circular, single-stranded RNA molecules without a protein coat. They exclusively infect plants, relying entirely...
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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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Related Experiment Video

Updated: Nov 1, 2025

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species
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[Prion diseases or transmissible spongiform encephalopathies].

J-P Brandel1

  • 1Cellule nationale de référence des maladies de Creutzfeldt-Jakob, Groupe hospitalier Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France; Inserm U1127/Institut du cerveau et de la moelle épinière (ICM), Groupe hospitalier Pitié-Salpêtrière, Centre national de référence des agents transmissibles non conventionnels, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.

La Revue De Medecine Interne
|June 21, 2021
PubMed
Summary

Prion diseases, or transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative conditions caused by infectious proteins called prions. Their unique nature as protein-only agents and links to genetic and infectious diseases make them a critical research area.

Keywords:
Creutzfeldt-Jakob diseaseEncéphalopathie spongiforme transmissibleFatal familial insomniaGerstmann-Straussler-ScheinkerInsomnie fatale familialeKuruMaladie de Creutzfeldt-JakobPrionTransmissible spongiform encephalopathiesVariant of Creutzfeldt-Jakob diseaseVariante de la maladie de Creutzfeldt-Jakob

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Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Genetics

Background:

  • Prion diseases, or transmissible spongiform encephalopathies (TSEs), are rare but fatal neurodegenerative disorders.
  • The causative agent is hypothesized to be an abnormal prion protein (PrPsc) that arises from a normal cellular protein (PrPc).
  • TSEs are characterized by specific brain pathologies including spongiosis, neuronal loss, and PrPsc deposits.

Purpose of the Study:

  • To review the nature of prion diseases and the proposed mechanism of prion formation.
  • To discuss the diagnostic significance of PrPsc detection.
  • To highlight the multifaceted nature of prion diseases, linking them to neurodegenerative, infectious, and genetic disease categories.

Main Methods:

  • Review of existing literature on prion diseases and transmissible spongiform encephalopathies.
  • Analysis of the proposed protein-only hypothesis for prion infectivity.
  • Examination of the neuropathological hallmarks and diagnostic criteria for TSEs.

Main Results:

  • Prion diseases are characterized by the misfolding of cellular PrP into protease-resistant PrPsc aggregates.
  • No immune or inflammatory responses are observed in TSEs, yet PrPsc presence is crucial for diagnosis.
  • The identification of various forms of CJD and related disorders underscores their complex etiology.

Conclusions:

  • Prion diseases represent a unique class of diseases caused by infectious proteins.
  • Understanding prion pathogenesis is vital due to their implications in neurodegeneration, public health, and genetics.
  • Prion diseases serve as a model for studying protein misfolding disorders and transmissible agents.