Prions: The Protein of Your Nightmares

Abstract

From the author, Passley Hargrove: Mad Cow disease was uncovered in Great Britain a decade ago, and was showcased worldwide on almost every single newspaper and station. The disease occurred when cattle were fed ground up cattle meal, which included brain tissue. The cows displayed a complex host of symptoms that ultimately led researchers to diagnose Mad Cow disease as a rare, neurological disorder known as bovine spongiform encephalopathy (BSE). Humans ingesting beef of cows with BSE developed many of the same neurological problems, and were diagnosed with Cruetzfeldt-Jacob's disease. Both diseases have been identified by the Center for Disease Control or CDC as having been caused by prions or protenacious infectious particles. These transmissible, virus-like agents cause parts of the brain to degenerate, by causing bubbles to form in brain cells. After sections of the brain dissolve holes are left reminiscent of a sponge. Prions cause progressive, neurodegenerative disorders in both animals and humans due to a mutation in the prion gene or in most cases, sporadically. Currently, the CDC and National Institute of Health are working on ways to cure these deadly diseases, however a remedy has yet to be found. In this literature review paper for Dr. Kjellerup's Microbiology and Immunology course, I compile information from various scientific sources that examine this rare family of diseases, paying close attention to the treatment strategies outdo by Phillip Yam, of Scientific American, in his book The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases. Prevention, accurate diagnosis, and treatment for prion diseases are of the upmost importance now.