Related Experiment Video
Updated: Feb 24, 2026
12:57
Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
16.6K
Infectious and Sporadic Prion Diseases.
1National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, United Kingdom.
Progress in Molecular Biology and Translational Science
|August 26, 2017
Summary
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder. This review focuses on sporadic and acquired CJD, including iatrogenic and variant forms (vCJD), and discusses genetic risk factors and diagnostic advancements.
Area of Science:
- Neurology
- Infectious Diseases
- Genetics
Background:
- Prion diseases, including Creutzfeldt-Jakob disease (CJD), are fatal neurodegenerative disorders.
- CJD presents in sporadic, acquired (iatrogenic, variant vCJD), and inherited forms.
- Sporadic CJD affects middle-aged and elderly populations, with PRNP-129 polymorphism as a key genetic risk factor.
Purpose of the Study:
- To review the sporadic and acquired forms of Creutzfeldt-Jakob disease (CJD).
- To discuss the epidemiology, risk factors, and transmission routes of iatrogenic CJD and variant CJD (vCJD).
- To highlight recent advancements in CJD diagnosis.
Main Methods:
- Review of literature on sporadic and acquired CJD.
- Analysis of epidemiological data and genetic risk factors (PRNP-129 polymorphism).
- Discussion of transmission pathways, including medical procedures, food contamination (BSE), and blood products.
Main Results:
- Sporadic CJD has recognized genetic risk factors, primarily the PRNP-129 polymorphism.
- Iatrogenic CJD is linked to cadaveric human growth hormone and dura mater grafts.
- Variant CJD (vCJD) originated from bovine spongiform encephalopathy (BSE) and has shown secondary transmission via blood transfusion.
Conclusions:
- The PRNP-129 polymorphism influences susceptibility and incubation periods for CJD.
- Concerns remain regarding secondary transmission of vCJD, particularly through blood products, with evidence of subclinical infections.
- Protein amplification techniques are improving clinical diagnosis of CJD, complementing pathological confirmation.
Related Concept Videos
01:29Subviral Agents
642
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...
642
03:03Amyloid Fibrils
12.1K
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,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
12.1K
03:03Amyloid Fibrils
6.8K
6.8K
01:28Fungal Phylum Microsporidia
600
Microsporidia are a group of obligate intracellular fungi that were initially classified as protists but were later reclassified based on phylogenetic, molecular, and structural evidence linking them to the Chytridiomycota. These unicellular, non-motile organisms are highly specialized parasites that infect a wide range of animal hosts, including humans. They have evolved extensive genomic and metabolic reductions, making them highly dependent on their hosts for survival.Morphology and Genomic...
600
01:30Bacterial Phylum Spirochaetes
948
Spirochetes, unique bacteria in the phylum Spirochaetes, are gram-negative, motile, tightly coiled, slender, and flexible. They inhabit aquatic sediments and animals, with some causing diseases like syphilis. Spirochetes are classified into eight genera based on habitat, pathogenicity, phylogeny, and characteristics.Their distinctive motility arises from endoflagella, located within the cell’s periplasm. These endoflagella anchor at the cell poles and extend along the cell length, encased...
948
01:20Infection
12.9K
When a pathogen enters the body and reproduces, it can cause an infection, damage body cells, and cause illness symptoms that eventually lead to disease. Therefore, its prevention requires breaking the chain of infection.
The chain begins with pathogens: bacteria, viruses, fungi, prions, or parasites such as protozoa helminths. These can be present on the skin as transient or resident flora, or they can be acquired from the environment. Identifying and treating the type of infection and...
The chain begins with pathogens: bacteria, viruses, fungi, prions, or parasites such as protozoa helminths. These can be present on the skin as transient or resident flora, or they can be acquired from the environment. Identifying and treating the type of infection and...
12.9K