Advanced Molecular Genetics-Biology 566 Prion Genetics: New Rules for a New Kind of Gene
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Prion Genetics: New Rules for a New Kind of Gene
Annual Review of genetics, 2004, 38:681-707. Reed B. Wickner, Herman K. Edskes, Eric D. Ross, Michael M. Pierce, Ulrich Baxa, Andreas Brachmann, and Frank Shewmaker

Amyloid

"Amyloid, which plays a large role in prion phenomena, is a protein assembly characterized by (a) filamentous morphology, (b) high beta sheet content with beta strands perpendicular to the fibril axis, (c) relative protease resistance, and (d) birefringence on staining withthe dye Congo Red."

There are over 20 disease-associated human proteins that reflect common structural features found in amyloids.

Four types of known prion

Transmissible Spongiform Encephalopathies

BSE in cattle, Scrapie in sheep, chronic wasting disease of deer and elk, Creuztfeldt-Jacob disease (CJD) in humans

Have a long incubation period (months to years)

Pathology only in the nervous system with accumulation of large vesicles, loss of neurons, proliferation of flial cells, absence of immunologic response.

Species Barriers slow incubation period but subsequent passage within a species shows shorter incubation time. Sinc gene related to incubation period.

Genetic criteria for prions of microorganisms