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Chapter 4

BIOL*1040 - Chapter 4

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BIOL 1020

Kaelan Parker 0744523 Prion Diseases A prion is a protein that has been misfolded during protein transcription. It is not know what exactly causes a protein to become misfolded, but once one becomes a prion, they can cause extremely serious disease and illness. Prions are the cause of diseases such as Creutzfeldt-Jakob disease, Kuru, and Mad Cow disease. Thee are just a few of the diseases that are cause by prion proteins. Creutzfeldt-Jakob disease, or CJD, is a rare disease that occurs hereditarily as a result of misfolded proteins. Only about 1 in a million people are affected by CJD a year. Symptoms of CJD include congnitive decline resulting in dementia, loss of muscle coordination, and myoclonus (Muscle twitching). CJD also usually results in death a few weeks after symptoms first appear (Aguzzi, Baumann, Bremer, 2008). Kuru was first discovered in the Fore people of Papa New Guinea in the 1950s. This Kuru epidemic was later found to be a result of cannibalistic rituals. The Fore people would eat the remains of the deceased. Women and children would eat the internal viscera and males would eat skeletal tissues (Collins, Lawson, Masters, 2004). Since Kuru is a disease caused a prion protein, it can be transmitted sporadically via cannibalism and infect the host with the Kuru disease. Just like Kuru
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