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

[Prion diseases].

T Billette de Villemeur1

  • 1Service de neuropédiatrie, hôpital Trousseau, 26, avenue Docteur-A.-Netter, 75012 Paris, France. thierry.billette@trs.ap-hop-paris.fr

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|June 24, 2003
PubMed
Summary
This summary is machine-generated.

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

Mortality in French people with polyhandicap/profound intellectual and multiple disabilities.

Journal of intellectual disability research : JIDR·2024
Same author

Impact of an intensive multimodal educative program on behavioral disorders of polyhandicapped patients: A randomized controlled trial.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2022
Same author

Clinical characteristics of COVID-19 infection in polyhandicapped persons in France.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2021
Same author

Development and initial validation of the polyhandicap severity scale.

Revue neurologique·2020
Same author

Health issues in polyhandicapped patients according to age: Results of a large French cross-sectional study.

Revue neurologique·2020
Same author

Health characteristics and health care trajectory of polyhandicaped person before and after 1990.

Revue neurologique·2019
Same journal

Family experiences of paediatric palliative care during hospital-at-home: a systematic review.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
Same journal

Knowledge of maternity caregivers on vaccination BCG recommendations and circuits.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
Same journal

"CRANIOQUALITY study: Quality of life in children with non-syndromic craniosynostosis after surgery".

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
Same journal

Chronological evolution of brain imaging of hemiconvulsion-hemiplegia-epilepsy from 5 cases in Mayotte island.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
Same journal

Point-of-care capillary blood ketone testing to predict the need for intravenous or nasogastric tube rehydration in children with acute gastroenteritis.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
Same journal

Transition practices of patients with inflammatory bowel disease from pediatric to adult healthcare systems. Results from a national survey.

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie·2026
See all related articles

Prion diseases arise from abnormal prion protein accumulation, leading to rare neurological disorders. Understanding prion biology enables effective preventive measures against juvenile forms.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Pathology

Context:

  • Prions are essential mammalian membrane proteins, primarily synthesized in the central nervous system.
  • Prion diseases result from the accumulation of misfolded prion proteins resistant to degradation and possessing infectivity.
  • Human prion diseases are rare, encompassing sporadic, familial, and iatrogenic forms, including variant Creutzfeldt-Jakob disease linked to bovine spongiform encephalopathy.

Purpose:

  • To elucidate the fundamental biology of prions and prion diseases.
  • To detail the various forms of human prion diseases and their origins.
  • To highlight the impact of prion biology knowledge on disease prevention.

Summary:

  • Prions, vital proteins in mammals, can misfold, resist degradation, and become infectious, causing neurodegenerative diseases.

Related Experiment Videos

  • Human prion diseases include sporadic Creutzfeldt-Jakob disease (presenile dementia), familial forms, and juvenile types like iatrogenic CJD and variant CJD (from BSE).
  • Advances in understanding prion biology have facilitated the development of preventive strategies, particularly for juvenile prion diseases.

    • Impact:

    • Enhanced understanding of prion pathogenesis.
    • Improved diagnostic and preventive strategies for rare neurological disorders.
    • Reduced incidence of iatrogenic and foodborne prion diseases through informed public health policies.