Prion Diseases
PATHOGENS
- Prions are abnormally folded versions of cell membrane proteins otherwise normally found throughout the human body.
- Abnormal versions are resistant to most types of proteolytic degradation, including resistance to many chemicals and physical methods of inactivation.
- The origin of initial conversion to the abnormal version is unclear but, once present, can induce the conversion of nearby proteins from the normal cellular prion protein (PrPC) form to the abnormal and infective prion scrapie (PrPSc) form.
- Normal PrPC and abnormal PrPSc forms share identical protein primary structures, so the abnormal PrPSc does not seem to trigger an immune response.
- Normal PrPC plays an important role in the organogenesis and development of the central nervous system; it also affects the pathogenesis of neurodegenerative diseases and cancers
- Prions cause transmissible spongiform encephalopathies (TSEs).
- Epidemiology: Annual incidence is about 1.2 cases per million people in the U.S.; it increases with age.
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Last updated: May 19, 2023
Citation
Crowell, Trevor A. "Prion Diseases." Johns Hopkins ABX Guide, The Johns Hopkins University, 2023. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/Johns_Hopkins_ABX_Guide/540451/all/Prion_Diseases.
Crowell TA. Prion Diseases. Johns Hopkins ABX Guide. The Johns Hopkins University; 2023. https://peds.unboundmedicine.com/pedscentral/view/Johns_Hopkins_ABX_Guide/540451/all/Prion_Diseases. Accessed October 15, 2024.
Crowell, T. A. (2023). Prion Diseases. In Johns Hopkins ABX Guide. The Johns Hopkins University. https://peds.unboundmedicine.com/pedscentral/view/Johns_Hopkins_ABX_Guide/540451/all/Prion_Diseases
Crowell TA. Prion Diseases [Internet]. In: Johns Hopkins ABX Guide. The Johns Hopkins University; 2023. [cited 2024 October 15]. Available from: https://peds.unboundmedicine.com/pedscentral/view/Johns_Hopkins_ABX_Guide/540451/all/Prion_Diseases.
* Article titles in AMA citation format should be in sentence-case
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