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 in pediatrics].

I Sol-Caubel1, F Castela, V Brousse

  • 1Service de neuropédiatrie, hôpital Armand-Trousseau, Paris, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|April 7, 1999
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

Crisis-like Seizure Exacerbations in NPRL3-related Epilepsy: Phenotypic Features and Treatment Outcomes.

Neuropediatrics·2025
Same author

<i>ARID1B</i>-related disorder in 87 adults: Natural history and self-sustainability.

Genetics in medicine open·2024
Same author

De Novo Balanced Translocations Disrupting the FBN1 Gene Diagnosed by Genome Sequencing: An Uncommon Cause of Marfan Syndrome Modifying Genetic Counseling.

American journal of medical genetics. Part A·2024
Same author

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

Journal of intellectual disability research : JIDR·2024
Same author

Identification of the first homozygous intragenic deletion in the YY1AP1 gene in a consanguineous family: New insights into the phenotypic variability associated with Grange syndrome.

American journal of medical genetics. Part A·2023
Same author

Cost of exome analysis in patients with intellectual disability: a micro-costing study in a French setting.

BMC health services research·2023
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, like Creutzfeldt-Jakob disease (CJD), involve abnormal prion protein (PrP) accumulation. Research explores CJD transmission, particularly variant CJD linked to bovine spongiform encephalopathy, and the role of the PRNP gene.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Molecular Biology

Context:

  • Prion diseases are rare, fatal neurological disorders affecting humans and animals.
  • Creutzfeldt-Jakob disease (CJD) is the most common pediatric prion disease, often linked to growth hormone treatment.
  • Variant CJD (vCJD) emerged in the UK, with strong evidence linking it to bovine spongiform encephalopathy (BSE).

Purpose:

  • To review the current understanding of prion diseases, focusing on CJD and vCJD.
  • To discuss the role of prion protein (PrP) accumulation and the PRNP gene in disease pathogenesis.
  • To address unanswered questions regarding potential new pediatric vCJD cases and transmission routes.

Summary:

  • Prion diseases are characterized by the accumulation of misfolded prion protein (PrP) in the brain.

Related Experiment Videos

  • The PRNP gene, encoding PrP, and its mutations/polymorphisms are implicated in CJD.
  • The link between BSE and vCJD highlights potential zoonotic transmission, raising concerns about dietary exposure.

    • Impact:

    • Highlights the need for further research into prion disease mechanisms and transmission.
    • Informs public health strategies for preventing and managing prion disease outbreaks.
    • Underscores the importance of understanding the PRNP gene's role in susceptibility and disease progression.