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

Transgenesis applied to transmissible spongiform encephalopathies.

Jean-Luc Vilotte1, Hubert Laude

  • 1Laboratoire de Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78352, Jouy-en-Josas Cedex, France. vilotte@jouy.inra.fr

Transgenic Research
|January 2, 2003
PubMed
Summary

Transmissible spongiform encephalopathies (TSEs) are fatal brain diseases caused by prions. Transgenic models are crucial for understanding prion diseases and developing therapies.

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

An autosomal recessive nonsense variant in the EGFR gene induces perinatal lethality in "Blonde d'Aquitaine" calves.

BMC veterinary research·2026
Same author

Overexpression of the STOX1B isoform of STOX1 triggers preeclampsia-like symptoms through HNF4α-dependent alterations of coagulation cascades in mice.

Scientific reports·2025
Same author

The prion-family protein Doppel exerts a protective role during influenza virus infection.

Journal of immunology (Baltimore, Md. : 1950)·2025
Same author

CRISPR/Cas9-editing of PRNP in Alpine goats.

Veterinary research·2025
Same author

Congenital microcoria deletion in mouse links Sox21 dysregulation to disease and suggests a role for TGFB2 in glaucoma and myopia.

American journal of human genetics·2024
Same author

A partial deletion within the meiosis-specific sporulation domain SPO22 of Tex11 is not associated with infertility in mice.

PloS one·2024

Area of Science:

  • Neuroscience
  • Prion Biology
  • Genetics

Background:

  • Transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases affecting mammals.
  • The 'protein-only' hypothesis posits prions (misfolded proteins) as the infectious agents.
  • The BSE crisis heightened concerns about human prion diseases like variant Creutzfeldt-Jakob disease (vCJD).

Purpose of the Study:

  • To review the application of transgenic models in studying TSEs.
  • To explore the role of host proteins in prion disease pathogenesis.
  • To investigate the genetic basis of the species barrier effect in prion transmission.

Main Methods:

  • Utilizing transgenic animal models to study prion protein function and disease.
  • Analyzing structure-function relationships of prion proteins.

Related Experiment Videos

  • Assessing genetic factors influencing species barriers.

    • Main Results:

    • Transgenic approaches have provided insights into prion protein's role in disease.
    • These models have helped elucidate the mechanisms behind the species barrier effect.
    • Transgenesis has compensated for the lack of in vitro models for prion diseases.

    Conclusions:

    • Transgenic experiments significantly advance the understanding of TSEs.
    • Continued research using transgenic models is vital for developing therapeutic strategies.
    • Further investigation is needed to address remaining questions about prion diseases.