Prion Diseases

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Prion Diseases

Abstract

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that are thought to be caused by the misfolding of prion proteins. Prions are able to replicate in the absence of nucleic acids. TSEs include: scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, kuru, Gerstmann-Straussler-Scheinker disease, and Fatal Familial Insomnia. They can affect many different animals, including humans. Currently, there are no ways to diagnose, treat, or cure TSEs, as much more research is needed before these diseases are completely understood.

1. Overview

Prions are a type of protein found naturally in the brain and other regions of the central nervous system. The diseases associated with prions are collectively known as transmissible spongiform encephalopathies (TSEs). “Transmissible” refers to their potentially infectious nature, and “spongiform encephalopathies” indicates the microscopic sponge-like deterioration of the brain caused by the progression of the disorders. While these fatal neurodegenerative diseases exhibit different clinical symptoms, have different incubation periods, and even target different areas of the brain, they do share a number of characteristics. They occur in both animals and humans. During a silent incubation period, there are no detectable signs of the disease, although depending on the specific disease, the length of the incubation period can “vary from a few weeks to up to 40 [years]” (Baker & Ridley, 1996, 1). Due to their unique method of propagation (which will be addressed later), TSEs present a seeming paradox in that “inherited cases give rise to a disease that is transmissible but acquired cases do not produce heritable diseases” (Baker & Rid...

... middle of paper ...

...(Soto, 2006, 105).

15. Conclusion

On a closing note, prion research is significant not only for possible breakthroughs in understanding TSEs, but because of the vast implications the very concept of prions holds for the entire field of biology. As the first substance discovered that can replicate in the absence of nucleic acids, prions defy one of the most central biological doctrines. The similarities between TSEs and dementia disorders like Alzheimer’s disease and “findings of proteins with a prion-like behavior in yeast and other fungi” (Soto, 2006, 143) suggest that prions and proteins like them may be much more common than ever expected (Soto, 2006, 154).

10

Bibliography

Baker, Harry F., & Ridley, Rosalind M. (1996). Prion Diseases. Totowa, New Jersey: Humana

Press.

Soto, Claudio. (2006). Prions: The New Biology of Proteins. Boca Raton, Florida: CRC Press.

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