Prion genetics: New rules for a new kind of gene

被引:61
作者
Wickner, RB [1 ]
Edskes, HK [1 ]
Ross, ED [1 ]
Pierce, MM [1 ]
Baxa, U [1 ]
Brachmann, A [1 ]
Shewmaker, F [1 ]
机构
[1] NIDDKD, NIH, Lab Biochem & Genet, Bethesda, MD 20892 USA
关键词
amyloid; URE3; PSI; Het-s; PIN; PrP;
D O I
10.1146/annurev.genet.38.072902.092200
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
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.
引用
收藏
页码:681 / 707
页数:31
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