Prions the Proteinacious Killer

Proteinacious Killer

Abstract:

Prions are proteins (PrP) that usually reside in the brain. There are two different forms of prions: normal PrPC and the pathological isoform, PrPSc. The two forms are chemically similar but shaped, or folded differently. PrPC occurs naturally in most mammals but PrPSc seems to bring with it various diseases, or spongiform encephalopathies. Various forms of these diseases have been found in many animal species, including humans. The actual means by which PrPSc propagates itself remains unknown but scientists believe the modified protein alters the normal protein molecules. The diseases may be contracted in a variety of ways. They may be genetically inherited, acquired by contamination, or occur sporadically on their own. All forms, however, are fatal.

The Prion Diseases:

Several diseases can result from prions. Such diseases are known as spongiform encephalopathies because they frequently cause the brain to “become riddled with holes”. The transmissible prion diseases are often called TSE (Transmissible Spongiform Encephalopathy) as well. All of these diseases are fatal (Prusiner). Though some of these diseases are well-known, like mad cow disease (also known as Bovine Spongiform Encephalopathy, or BSE), others are even more sinister. Many prion diseases exist, affecting several animal species, including humans. In all cases however, “the disease is either naturally sporadic, genetically determined, or acquired by contamination” (Ridley, 9).

The Human Diseases:

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Creutzfeldt - Jakob disease (also known as CJD) is a human disease that may be inherited (genetically determined), contracted through contamination, or occur sporadically. New variant CJD is thought to result from eating food "cont...

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...ning the TSE infectious agent. PrPSc seems to convert normal PrPC molecules to its altered form, spreading disease. Many prion diseases exist, affecting several animal species, including humans. They may be contracted through various means. However, whether a sporadic occurrence, an acquired attribute, or a genetic inheritance, all prion diseases are fatal. Sometimes the cause does not matter; in the end everyone dies.

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Works Cited

Graham, Sarah. "New Form of Mad Cow Protein Discovered." Scientific American (2004). 23 July 2008 .

Prusiner, Stanley B. "The Prion Diseases." 23 July 2008 .

Ridley, Rosalind M., and Harry F. Baker. Fatal Protein. New York: Oxford UP, Inc., 1998.

Soto, C. (2006). Prions: The New Biology of Proteins. Taylor & Francis Group: CRC Press.