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PRIONS: MYSTERIOUS INFECTIOUS AGENT

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Most of us are familiar with most diseases and their causes. Some, like the common cold, are most likely caused by a virus. Bacteria cause other illnesses, such as botulism and tuberculosis; protozoans cause malaria, African Sleeping Sickness, amoebic dysentery and more. In general, most infectious pathogenic illnesses are caused by microorganisms.

However, prion is an agent that causes a series of fatal diseases in humans and a few other animals. The reason why prions are a cause for concern is the fact that antibiotics or antiviral medications are ineffective — the reason why none of these medications work is because prions are technically not a living organism.

Priors are healthy proteins that happen to “misfold”. These misfolded proteins also tend to misfold other healthy proteins in the organism. Kuru disease is a sporadic prion disease that affects the tissues in the human brain. If contracted, the disease is fatal and has no known cure. One of the characteristics of the disease is that many tiny holes appear in the cerebral cortex of the brain – which is reminiscent of a sponge.

When an individual contracts this disease, they initially develop behavioural and personality changes. Problems with movement and locomotion also set in, with worsen over time. Towards the end of the terminal stage, the patient develops ulcers that can become easily infected. Usually, after an individual exhibits their first terminal stage symptoms, they succumb to the disease within three months to two years.

Prions also cause disease in cattle – with the most infamous being the mad-cow disease. Signs are not immediately noticeable due to the extremely long incubation period of the disease – typically 4 to 5 years. However, typical signs of the disease include unexplained weight loss, decreased milk production, ear infections, changes in temperament, aggression and abnormal gait. These signs can eventually lead to coma and death of the animals within weeks to months. If the infected animal is consumed, the disease can pass on to humans and result in a variation of the Creutzfeldt-Jakob Disease.

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