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, update].

T Kitamoto1

  • 1Department of Neurological Science, Tohoku University School of Medicine.

Rinsho Shinkeigaku = Clinical Neurology
|September 19, 2002
PubMed
Summary
This summary is machine-generated.

Fragmented prion protein (PrP) molecules offer a new way to classify human prion diseases, aiding in understanding disease subtypes and transmission. This discovery helps differentiate specific prion disease types and analyze abnormal PrP structures.

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

Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease.

European journal of neurology·2016
Same author

Actions of the Japanese Pancreas and Islet Transplantation Association regarding transplanted human islets isolated using Liberase HI.

Transplantation proceedings·2010
Same author

Survival to akinetic mutism state in Japanese cases of MM1-type sporadic Creutzfeldt-Jakob disease is similar to Caucasians.

European journal of neurology·2010
Same author

Discordant clinicopathologic phenotypes in a Japanese kindred of fatal familial insomnia.

Neurology·2009
Same author

Less protease-resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate.

Acta neurologica Scandinavica·2009
Same author

Changes in expression of sensory organ-specific microRNAs in rat dorsal root ganglia in association with mechanical hypersensitivity induced by spinal nerve ligation.

Neuroscience·2009

Area of Science:

  • Neuroscience and Neurology
  • Biochemistry and Molecular Biology

Context:

  • Prion diseases, including Creutzfeldt-Jakob disease (CJD), exhibit diverse clinical and pathological phenotypes despite shared prion protein (PrP) genotypes.
  • Existing classification methods based on PrP genotype alone are insufficient to capture the full spectrum of disease variability.
  • Recent advancements include PrP typing based on the molecular weight of protease-resistant PrP (PrPres), offering more precise classification.

Purpose:

  • To investigate the utility of fragmented prion protein (PrP) molecules as a marker for classifying human prion diseases.
  • To analyze the relationship between fragmented PrP, PrP genotype, PrPres typing, and clinicopathological phenotypes.
  • To explore the role of fragmented PrP in differentiating specific CJD subtypes and understanding prion transmissibility.

Related Experiment Videos

Summary:

  • This study identifies fragmented PrP molecules as a novel and useful marker for classifying human prion diseases, including various forms of CJD and Gerstmann-Straussler syndrome (GSS).
  • Fragmented PrP successfully differentiated between dura-classic CJD and dura-plaque type CJD, and its presence correlated with successful prion transmission in sporadic CJD cases with type 1 PrPres.
  • The findings suggest that fragmented PrP provides crucial insights into abnormal PrP structures, contributing to clinicopathology and transmissibility in prion diseases.

Impact:

  • Establishes fragmented PrP as a valuable diagnostic and classification tool in human prion diseases.
  • Enhances understanding of the structural variations in abnormal PrP and their impact on disease progression and infectivity.
  • Provides a foundation for further research into the mechanisms underlying prion disease heterogeneity and transmissibility.