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 Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Subviral Agents01:29

Subviral Agents

Subviral agents are infectious entities that resemble viruses but lack one or more viral components, such as a capsid or essential replication machinery. These agents include viroids, prions, and satellites, each possessing distinct structural and functional characteristics that influence their mode of infection and replication.Viroids are the simplest subviral agents, consisting of circular, single-stranded RNA molecules without a protein coat. They exclusively infect plants, relying entirely...
Rabies01:28

Rabies

Rabies is a lethal zoonotic disease caused by a single-stranded, negative-sense RNA virus of the Lyssavirus genus, within the family Rhabdoviridae. Its primary mode of transmission to humans is through bites or saliva-contaminated scratches from infected mammals such as dogs, bats, raccoons, or foxes. Transmission can also occur if infectious saliva contacts abraded skin or intact mucous membranes, including the conjunctiva.Viral Entry and Early ReplicationOnce introduced at the bite or scratch...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
Plague01:24

Plague

Plague is a highly virulent zoonotic disease caused by Yersinia pestis, a Gram-negative, facultatively anaerobic coccobacillus. This pathogen primarily circulates among rodent populations and is transmitted to humans through the bite of infected fleas. Additional transmission routes include direct contact with infected animal tissue or inhalation of respiratory droplets from individuals with pneumonic plague. These multiple transmission pathways highlight the bacterium’s potential for rapid...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Nationwide screening identifies an expanded clinical spectrum of anti-KLHL11 antibody-associated neurologic disease in Japan.

Journal of neurology·2026
Same author

Prevalence and profiles of clinically diagnosed autoimmune cerebellar ataxia in a Japanese nationwide survey.

Journal of neurology·2026
Same author

A noninvasive test for human prion disease using hair roots and scalp.

Scientific reports·2025
Same author

A Case of CACNA1I-Related Neurodevelopmental Disorder With Dysmorphism and Brain Iron Accumulation: Expanding the Clinical Spectrum.

Clinical genetics·2025
Same author

Isoform analysis of heterozygous putative splicing variants at the allele level using nanopore long-read sequencing.

Scientific reports·2025
Same author

Spinocerebellar ataxia type 2 followed by amyotrophic lateral sclerosis due to a pure CAG repeat expansion in ATXN2: a case report and literature review.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·2025

Related Experiment Video

Updated: May 29, 2026

Prion Safety Laboratory Swipe Test
06:01

Prion Safety Laboratory Swipe Test

Published on: February 14, 2025

[Prion disease].

Hidehiro Mizusawa1

  • 1Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Graduate School of Medical and Dental Sciences.

Rinsho Shinkeigaku = Clinical Neurology
|September 17, 2011
PubMed
Summary

Human prion diseases, primarily sporadic Creutzfeldt-Jakob disease (sCJD), are identified through surveillance. Diagnostic tools like MRI and CSF analysis are crucial for detecting these rare, fatal neurological disorders.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Genetics

Context:

  • Human prion diseases encompass idiopathic, acquired, and genetic forms.
  • Surveillance identified 1,402 prion disease cases from 2,494 analyzed.
  • Sporadic CJD constitutes the majority (77%), followed by genetic (17%) and acquired (5%).

Purpose:

  • To analyze prion disease surveillance data in Japan.
  • To highlight diagnostic challenges and characteristic findings.
  • To discuss unique genetic mutations and acquired forms.

Summary:

  • Mean age of onset is late 60s; characteristic signs include brain MRI and elevated CSF 14-3-3 and tau proteins.
  • Sporadic CJD (MM1) is common in Japan, but atypical subtypes like MM2-thalamic CJD pose diagnostic difficulties.

More Related Videos

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species
12:00

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species

Published on: November 6, 2013

Related Experiment Videos

Last Updated: May 29, 2026

Prion Safety Laboratory Swipe Test
06:01

Prion Safety Laboratory Swipe Test

Published on: February 14, 2025

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species
12:00

Procedures for Identifying Infectious Prions After Passage Through the Digestive System of an Avian Species

Published on: November 6, 2013

  • Genetic prion diseases in Japan feature unique mutations (V180I, M232R), and dura mater graft-associated CJD shows varied phenotypes.

    • Impact:

    • Diagnostic tools like EEG, MRI, genetic, CSF tests, and SPECT are vital for most prion cases.
    • Understanding atypical forms and potential iatrogenic transmission is critical.
    • International cooperation is essential for managing this intractable disease.