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

Human prion diseases.

M Haltia1

  • 1Department of Pathology, University of Helsinki and the Helsinki University Central Hospital, Finland. matti.j.haltia@helsinki.fi

Annals of Medicine
|November 22, 2000
PubMed
Summary
This summary is machine-generated.

Prion diseases, or transmissible spongiform encephalopathies, are fatal neurodegenerative disorders caused by prions. This review covers prion biology and key human prion disease features.

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

A prospective radiologic and neurologic follow-up study of 61 HIV-1 -infected subjects: early beginning and slow progression of brain atrophy.

European journal of neurology·2013
Same author

Cutis laxa in hereditary gelsolin amyloidosis.

The British journal of dermatology·2005
Same author

The normal population distribution of PRNP codon 129 polymorphism.

Acta neurologica Scandinavica·2003
Same author

Status epilepticus induces changes in the expression and localization of endogenous palmitoyl-protein thioesterase 1.

Neurobiology of disease·2002
Same author

[Year 2001 Matti Ayrapää award to Riitta Hari].

Duodecim; laaketieteellinen aikakauskirja·2002
Same author

Contribution of APOE promoter polymorphisms to Alzheimer's disease risk.

Neurology·2002
Same journal

Early metabolic trajectory and sedation strategy in V-V ECMO for severe pneumonia: a multicenter cohort study.

Annals of medicine·2026
Same journal

Analysis of the current status and influencing factors of influenza vaccination among medical staff in multiple regions of China.

Annals of medicine·2026
Same journal

Predictive value of gastrointestinal decompression volume and its trajectory over time for surgical intervention in children with postoperative adhesive small bowel obstruction.

Annals of medicine·2026
Same journal

Effects of blood flow restriction training combined with resistance training on lower-limb strength and sport-specific performance in athletes: a systematic review and meta-analysis.

Annals of medicine·2026
Same journal

Association between maternal serum RANKL and adverse birth outcomes: a prospective cohort study.

Annals of medicine·2026
Same journal

<i>Helicobacter pylori</i> in oral and gastric pathologies: a narrative review of potential bidirectional pathogenic interactions.

Annals of medicine·2026
See all related articles

Area of Science:

  • Neurology
  • Pathology
  • Infectious Diseases

Background:

  • Prion diseases are fatal neurodegenerative disorders.
  • Also known as transmissible spongiform encephalopathies (TSEs).
  • Caused by prions, unique infectious agents.

Purpose of the Study:

  • To review the basic biological properties of prions.
  • To present clinical and pathological features of human prion diseases.
  • To discuss the connection between variant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE).

Main Methods:

  • Literature review of prion diseases.
  • Discussion of prion replication and transmission.
  • Presentation of neuropathological changes in the central nervous system.

Related Experiment Videos

Main Results:

  • Prion diseases have long incubation periods and are invariably fatal.
  • Key human prion diseases include Creutzfeldt-Jakob disease (CJD), with sporadic, inherited, and infectious forms.
  • Bovine spongiform encephalopathy (BSE) in cattle and scrapie in sheep are significant animal counterparts.

Conclusions:

  • Understanding prion biology is crucial for diagnosing and managing these rare diseases.
  • The potential for human blood contamination by vCJD necessitates further research and vigilance.
  • Continued study of prion diseases is essential due to their fatal nature and potential public health implications.