Prion Diseases

Diseases, one of the most challenging problems one will ever face, can be extremely agonizing whether if one is suffering the sickness him or herself or watching a loved one suffer. Medical advancements form improved surgical procedures to life saving organ transplants, from disease eradicating vaccines to antibiotics have made life considerably easier for afflicted people. However, not even the most recent medical technology can save one suffering from a particular type of disease. This type of illness causes its victim’s health to rapidly deteriorate until the patient is delirious, bedridden, and as helpless as a baby. What’s worse is that this type disease always ends in death. This type of disease is called transmissible spongiform encephalopathy and it is not caused by common pathogens like viruses and bacteria. The cause is prions, small bundles of proteins that wasn’t discovered until the 80s. By looking at how prions cause disease, the diseases it causes, and the research being done, one can see epidemiology is a developing field of science.

To understand the diseases prions cause, one must understand how they cause disease. The PRNP gene code for healthy prion protein, PrPC, which has a helical structure. The role they play in our bodies is uncertain. When the PRNP gene is mutated, PrPSc protein are produced instead. However, PrPSc resembles a piece of string. Using a process not understood by scientists, PrPSc prions bind to healthy protein which mutates. Prions are extremely resistant to protease, enzymes that break down proteins into amino acids, causing a buildup of prions within a neuron. Eventually, the neuron explodes releasing prions which mutates additional proteins and leaving a small hole behind. As more neuro...

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... a team of scientists including Dr. Daniel Carleton Gadjusek arrived at New Guinea to study kuru. However, none could find a cause. However, Dr. Gadjusek noticed something strange about the Foré people; they were cannibals. When someone died, the Foré would participate in a ritualistic cannibalistic feast where instead of burying the deceased, they would consume them. Dr. Gadjusek tested by injecting kuru brains into monkeys. After a while, the monkeys caught kuru. Dr. Gadjusek concluded the infectious something lived in the brain. In addition, Gadjusek identified three stages in the disease. The first stage, the ambulant stage, resulted in the patient suffering from unsteadiness, speaking issues, and shivering. Next, the sedentary stage caused the patient to become unable to walk, losing control of muscles. Also, the victims begin to jerk and laugh uncontrollably.