Related Experiment Video
Updated: May 1, 2026
High-throughput Screening for Protein-based Inheritance in S. cerevisiae
Published on: August 8, 2017
Prions: a model of conformational disease?
1Pôle Biologie-Pathologie-Physiologie, Université Paris Diderot-Paris Sorbonne Cité, Groupe Hospitalier Saint-Louis-Lariboisière - Fernand-Widal, Assistance publique-Hôpitaux de Paris, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Abstract:
The discovery that a protein could mimic viral and bacterial pathogens around 1980 by Stanley Prusiner was unexpected. Evidence shows now that Creutzfeldt-Jakob disease and related disorders are caused by prions. Prions and, for example neurodegeneratives diseases, arise from the same general disease mechanism. In each, there is abnormal unfolding and then aggregation of proteins. The protein conformational changes associated with the pathogenesis of protein misfolding disorders produce β sheet rich oligomers that are partially resistant to proteolysis and have a high tendency to form amyloid-like aggregates. It is important to distinguish between prions and amyloids: prions need not to polymerize into amyloid fibrils and can undergo self-propagation as oligomers. The prion diseases are characterized by the conformational conversion of PrP(c) to PrP(sc), the fundamental even underlying prion diseases. Despite the obvious differences between prions and conventional infectious microorganisms, prions fulfill the Koch's postulates. Meaningful treatments are likely to require cocktails of drugs that interfere with the conversion of precursor into prions and enhance the clearance of prions; such an approach may find application in the more common degenerative diseases.
Insights
Prions, infectious proteins causing diseases like Creutzfeldt-Jakob disease, share mechanisms with neurodegenerative disorders. Understanding protein misfolding is key to developing treatments for these conditions.
Area of Science:
- Neurology
- Biochemistry
- Infectious Diseases
Background:
- Prions, discovered by Stanley Prusiner, are proteins that mimic pathogens.
- Creutzfeldt-Jakob disease and related disorders are caused by prions.
- Prions share disease mechanisms with neurodegenerative diseases, involving protein misfolding and aggregation.
Purpose of the Study:
- To explain the mechanism of prion diseases.
- To differentiate prions from amyloids.
- To suggest potential therapeutic strategies for prion and neurodegenerative diseases.
Main Methods:
- Review of scientific evidence on prion pathogenesis.
- Analysis of protein conformational changes in disease.
- Comparison of prion diseases with conventional infectious diseases and other neurodegenerative disorders.
Main Results:
- Prions cause Creutzfeldt-Jakob disease and related disorders.
- Protein misfolding disorders, including prion diseases and neurodegenerative conditions, share a common pathogenic mechanism.
- Prions are characterized by the conformational conversion of PrP(c) to PrP(sc) and can propagate as oligomers, not necessarily requiring amyloid polymerization.
- Prions fulfill Koch's postulates, despite differences from conventional infectious agents.
Conclusions:
- Prion diseases and neurodegenerative disorders stem from similar protein misfolding and aggregation mechanisms.
- Effective treatments may involve drug cocktails targeting prion conversion and clearance.
- This therapeutic approach could be applicable to common degenerative diseases.
Related Concept Videos
03:03Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
03:03Amyloid Fibrils
01:43Incomplete Dominance
01:31Translation
Translation Produces the Building Blocks of Life
Proteins are...
01:31Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
Translation Produces the Building Blocks of...
01:35Pedigree Analysis