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 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.
Wickner
Reed B
RB
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
Edskes
Herman K
HK
Ross
Eric D
ED
Pierce
Michael M
MM
Baxa
Ulrich
U
Brachmann
Andreas
A
Shewmaker
Frank
F
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
D000682:Amyloid; D018832:Molecular Chaperones; D011328:Prions