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Prions--infectious pathogens causing the spongiform encephalopathies
CRC Critical Reviews in Clinical Neurobiology
|January 1, 1985
Summary
Novel infectious agents, prions, cause scrapie and Creutzfeldt-Jakob disease (CJD). A specific gene, prion incubation determinant (PID), influences disease progression and is located on chromosome 17 in mice.
Area of Science:
- Neurodegenerative diseases
- Transmissible spongiform encephalopathies
- Molecular biology
Background:
- Scrapie and Creutzfeldt-Jakob disease (CJD) are caused by unique transmissible agents termed prions.
- Prions differ from viruses and viroids due to their novel properties.
- A protein essential for infectivity has been identified within the scrapie prion.
Purpose of the Study:
- To investigate the nature of prion diseases, including scrapie and CJD.
- To identify the genetic factors influencing prion disease incubation periods.
- To determine the chromosomal location of genes associated with prion disease.
Main Methods:
- Biochemical purification of prion components.
- Genetic analysis in congenic mouse models.
- Mapping of the prion incubation determinant (PID) gene.
Main Results:
- A 27,000-30,000 MW protein co-purifies with infectious prions.
- The prion incubation determinant (PID) gene, an autosomal dominant gene, controls disease time courses for both scrapie and CJD.
- In CJD-infected mice, PID is localized to chromosome 17 within the H-2 D-subregion.
Conclusions:
- Scrapie and CJD are likely caused by prions, with CJD sharing similarities with scrapie.
- Prion replication precedes pathological changes in the brain.
- The identified genetic locus (PID) on chromosome 17 provides a target for understanding prion disease pathogenesis and may offer insights into other degenerative neurological disorders.