Prion Diseases

Prions are malfunctioning proteins with the ability to transmit their misfolded shape onto normal variants of the same protein. They characterize several lethal and infective neurodegenerative diseases in humans and many other animals.

Prions cause neurodegenerative disease by accumulating extra cellular within the central nervous system to form plaquette known as amyloids, which disrupt the normal tissue shape. This disruption is characterized by "holes" in the tissue with resultant spongy architecture due to the vacuole formation in the neurons.

While the incubation period for prion diseases is relatively long about 5 to 20 years, once symptoms appear the disease progresses rapidly, leading to brain damage and death.

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