title: Prion genetics: new rules for a new kind of gene.
abstract: 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.
authors.affiliation: Laboraory of Biochemistry and Genetics, National Institute of Diabetes Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-0830, USA. wickner@helix.nih.gov
authors.forename: Reed B
authors.initials: RB
authors.lastname: Wickner
authors.forename: Herman K
authors.initials: HK
authors.lastname: Edskes
authors.forename: Eric D
authors.initials: ED
authors.lastname: Ross
authors.forename: Michael M
authors.initials: MM
authors.lastname: Pierce
authors.forename: Ulrich
authors.initials: U
authors.lastname: Baxa
authors.forename: Andreas
authors.initials: A
authors.lastname: Brachmann
authors.forename: Frank
authors.initials: F
authors.lastname: Shewmaker
mesh_terms: D000682:Amyloid / Q000737:chemistry / Q000235:genetics; D000818:Animals; D006224:Cricetinae; D006801:Humans; D051379:Mice; D008954:Models, Biological; D018832:Molecular Chaperones; D010641:Phenotype; D048468:Podospora / Q000235:genetics / Q000378:metabolism; D017096:Prion Diseases / Q000235:genetics; D011328:Prions / Q000235:genetics* / Q000378:metabolism; D011485:Protein Binding; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000378:metabolism; D015003:Yeasts / Q000235:genetics / Q000378:metabolism
chemical_list: D000682:Amyloid; D018832:Molecular Chaperones; D011328:Prions