Prusiner's Prion Discovery: One Man's Dedication - Essay Example

Though the discovery and subsequent prize was a major breakthrough, it presented as many questions as it answered. Along with the unanswered questions were critics who believed the work was incomplete and that the Nobel Prize may have been immature. However, nine years after the presentation of the award, the work continues to stand out and had opened up new avenues of investigation for the causes of many diseases that had until recently been shrouded in mystery. The work on the prion dates back to the 1960s when radiation biologist Tikvah Alper and physicist J.S. Griffith were studying the diseases of Transmissible spongiform encephalopathies (TSE) and scrapie in animals ("Prion").

They had noted that the infectious agents were resistant to ultraviolet radiation which had previously believed would kill all living organisms. These diseases, found in cows and sheep, were closely related to Creutzfeldt-Jakob Disease (CJD), a neurological disorder found in humans. It led to a crisis in France and Britain in the last twenty years where they were forced to kill tens of thousands of animals to prevent the spread of mad cow disease ("Transmissible Spongiform Encephalopathies").

Though these disorders in humans are very rare, they do affect about one person per million and are almost always fatal. Though the mechanism is not well understood, Belay and Schonberger state, "[.] the disease associated proteins may damage neurons directly, and diminished availability of the normal prion protein may interfere [.] with the underlying neurodegenerative process" (191). Prion diseases are difficult to diagnose and have long incubation periods, which further complicated the research.

Prusiner faced this difficulty as he was "[.] forced to use scores of mice and in each experiment wait patiently for approximately 200 days for the appearance of disease symptoms" ("Nobel Prize to Prusiner!"). Overcoming these obstacles to isolate the cause and discover the agent of these mysterious illnesses would gain Prusiner the Nobel Prize for Medicine in 1997. Stanley B. Prusiner became interested in the disease while he was working as an intern and one of his patients died Creutzfeldt-Jakob disease (CJD) and its resulting brain disorder.

At this time, he found little research that could be helpful in explaining and treating CJD. Prusiner recalls in his autobiography, "The more that I read about CJD and the seemingly related diseases - kuru of the Fore people of New Guinea and scrapie of sheep - the more captivated I became" (Prusiner). He embarked on a dedicated research program and within ten years his team had "[.] found an unusual protein in the brains of scrapie-infected hamsters that did not seem to be present in healthy animals" (Vogel 214).

Soon they would identify a protein, called PrP for prion protein, and over the course of the next ten years they would demonstrate that prions (PrP) are a common protein that is present in all animals, yet in diseased animals it took a different form (Vogel 214). This mystery would be the leading question that would unlock the mystery of the prion-based diseases.Prusiner's research was able to demonstrate that the common PrP protein could mutate into an agent that could cause scrapie, mad cow disease, CJD, and other neurological diseases. To