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Prions Disease Summary

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Prions Disease Summary- Alex Schray
Causes:
Prion diseases are caused by proteinaceous infectious particles (more commonly referred to as prions). According to the CDC the term prion “refers to abnormal, pathogenic agents that are transmissible and are able to induce abnormal folding of specific normal cellular proteins.” Prions are always contracted one of three different ways. They can develop sporadically through a sudden genetic mutation. They can be inherited; prions are autosmol dominant, meaning that children of the infected have a fifty percent chance of inheriting the disease. Finally, prions can be ingested: either through consumption of infected meat or after exposure to poorly cleaned medical instruments.
Symptoms: Creutzfeldt-Jakob
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In prions, however, the PRP primary structure remains the same but the secondary structure does not. The alpha helices which typically comprise most of the secondary structure are replaced by beta pleated sheets. This structural mutation makes prions especially dangerous. The increased number of beta sheets means prions cannot to be broken down by protease and thus have the un-prohibited ability to convert other proteins to prions. This mass conversion eventually causes holes to appear in the brain as grey matter deteriorates. This degeneration of the brain results in extreme changes in the nervous and muscular systems. As the brain develops holes from the prions, all function controlled by the brain begins to malfunction. Movement for example, controlled by and large by the cerebellum, becomes extremely difficult and disjointed when one has CJD- because of the deterioration of the grey matter of the cerebellum. The hippocampus, responsible for memories, loses its hippocampal grey matter as prions convert PRP molecules, causing the largescale loss of
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