NR ARUJ
AU Wickner,R.B.; Edskes,H.K.; Ross,E.D.; Pierce,M.M.; Baxa,U.; Brachmann,A.; Shewmaker,F.
TI Prion genetics: new rules for a new kind of gene.
QU Annual Review of Genetics 2004; 38: 681-707
PT journal article; review
AB 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.
ZR 139
MH Amyloid/chemistry/genetics; Animals; Hamsters; Humans; Mice; Models, Biological; Molecular Chaperones; Phenotype; Podospora/genetics/metabolism; Prion Diseases/genetics; Prions/*genetics/metabolism; Protein Binding; Saccharomyces cerevisiae/genetics/metabolism; Yeasts/genetics/metabolism
AD 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
SP englisch
PO USA