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Animation: Prions: Characteristics

by Pearson
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All mammalian cells contain a gene that codes for the primary sequence of amino acids for the prion protein, PrP. Normally, PrP folds into a functional form with several α-helices, called cellular PrP. The normal function of the prion protein is not well understood, but it is believed to be important in normal synaptic development and function, and it may be involved in stabilizing the structure of the synapses and in establishing memory. The prion protein is also capable of folding into a form with β-pleated sheets. Prions do not reproduce like bacteria or viruses. Instead, infectious prions convert normal prions into more infectious prions by folding them into beta-pleated sheets. Thus, the number of infectious prions has increased, but by conversion, rather than traditional reproduction. Prion proteins in the infectious conformation can group into multimers, which are very stable and resistant to protease. Over time, these multimers probably lead to some of the damage seen in prion-infected tissues.
All mammalian cells contain a gene that codes for the primary sequence of amino acids for the prion protein, PrP. Normally, PrP folds into a functional form with several α-helices, called cellular PrP. The normal function of the prion protein is not well understood, but it is believed to be important in normal synaptic development and function, and it may be involved in stabilizing the structure of the synapses and in establishing memory. The prion protein is also capable of folding into a form with β-pleated sheets. Prions do not reproduce like bacteria or viruses. Instead, infectious prions convert normal prions into more infectious prions by folding them into beta-pleated sheets. Thus, the number of infectious prions has increased, but by conversion, rather than traditional reproduction. Prion proteins in the infectious conformation can group into multimers, which are very stable and resistant to protease. Over time, these multimers probably lead to some of the damage seen in prion-infected tissues.