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biology of prions Dr. Stanley Prusiner coined the term prion in 1982, which he defined as a small infectious pathogen containing protein but apparently lacking nucleic acid . The prion protein (PrP) is the critical component of these agents and may, in fact, be its exclusive constituent.
The abnormal protein builds up in the brain, forming clumps that damage or destroy neurons. The loss of these cells creates microscopic sponge-like holes (vacuoles) in the brain, which leads to the signs and symptoms of prion disease.