Prion genetics: new rules for a new kind of gene

Just as nucleic acids can carry out enzymatic reactions, proteins can be genes. These heritable infectious proteins (prions) follow unique genetic rules that enable their identification: reversible curing, inducible "spontaneous generation," and phenotype surprises. Most prions are based o...

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Veröffentlicht in:Annual review of genetics 2004-01, Vol.38 (1), p.681-707
Hauptverfasser: Wickner, Reed B, Edskes, Herman K, Ross, Eric D, Pierce, Michael M, Baxa, Ulrich, Brachmann, Andreas, Shewmaker, Frank
Format: Artikel
Sprache:eng
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Zusammenfassung:Just as nucleic acids can carry out enzymatic reactions, proteins can be genes. These heritable infectious proteins (prions) follow unique genetic rules that enable their identification: reversible curing, inducible "spontaneous generation," and phenotype surprises. Most prions are based on self-propagating amyloids, depend heavily on chaperones, show strain phenomena and, like other infectious elements, show species barriers to transmission. A recently identified prion is based on obligatory self-activation of an enzyme in trans. Although prions can be detrimental, they may also be beneficial to their hosts.
ISSN:0066-4197
1545-2948
DOI:10.1146/annurev.genet.38.072902.092200