Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Pathogenesis of prion diseases.

Ursula Unterberger1, Till Voigtländer, Herbert Budka

  • 1Institute of Neurology, Medical University of Vienna, AKH, Austria.

Acta Neuropathologica
|January 13, 2005
PubMed
Summary

Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins. Research is advancing understanding of prion spread and nerve cell damage, but therapies are still needed.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

The challenge of persistent physical symptoms.

The British journal of general practice : the journal of the Royal College of General Practitioners·2025
Same author

In memoriam Prof. Pawel P. Liberski, MD PhD (November 25, 1954-August 19, 2025).

Acta neuropathologica·2025
Same author

Multiomic analyses direct hypotheses for Creutzfeldt-Jakob disease risk genes.

Brain : a journal of neurology·2025
Same author

Neuropathology in 1984: a deadly shot into the heart of Europe.

Free neuropathology·2025
Same author

Behind the scenes of EQA–characteristics, capabilities, benefits and assets of external quality assessment (EQA): Part IV – Benefits for participant laboratories

Clinical chemistry and laboratory medicine·2025
Same author

Neuropathological spectrum of anti-IgLON5 disease and stages of brainstem tau pathology: updated neuropathological research criteria of the disease-related tauopathy.

Acta neuropathologica·2024

Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Prion diseases are rare, fatal neurological disorders.
  • They can be genetic, infectious, or sporadic.
  • Prions are abnormal, protease-resistant prion proteins (PrP(C)) that aggregate in the central nervous system (CNS).

Purpose of the Study:

  • To review the pathogenesis of prion diseases.
  • To discuss peripheral and central mechanisms of prion disease.
  • To highlight the need for therapeutic development.

Main Methods:

  • Literature review of prion disease pathogenesis.
  • Analysis of prion replication and spread.
  • Examination of mechanisms of neurodegeneration in prion diseases.

Main Results:

  • Prion diseases involve aggregates of misfolded prion proteins in the CNS.
  • Peripheral pathogenesis involves prion replication in lymphoid tissues before CNS spread.
  • Central pathogenesis includes prion spread within the CNS and mechanisms of nerve cell damage.

Conclusions:

  • Understanding prion disease pathogenesis requires studying both peripheral and central mechanisms.
  • Lymphoid tissues play a key role in peripheral prion replication.
  • While microglial neurotoxicity, oxidative stress, and complement activation are implicated, further research is needed to develop effective therapies for prion diseases.

Related Experiment Videos