NR AEBA
AU Fisher,E.; Telling,G.; Collinge,J.
TI Prions and the prion disorders
QU Mammalian Genome 1998 Jul; 9(7): 497-502
PT journal article; review; review, academic
AB One of us remembers sitting in a high school biology class in 1977 being taught about scrapie, a naturally occurring disorder of sheep. The teacher had no particular interest in agriculture, but was pointing out some peculiar characteristics of this disease as a biological curiosity on a wet Friday afternoon. The prion disorders captured the imagination of a range of biologists (including that teacher) well before the epidemic of bovine spongiform encephalopathy (BSE) and the appearance of a new variant of the human prion disease, Creutzfeldt Jakob disease (CJD), in the UK, because of their extraordinary biology and the unique properties of the infectious agent. We review the results of studies leading to a convergence of evidence that the causative infectious agent, the 'prion', is devoid of nucleic acid and is composed of an abnormal isoform of a host-encoded protein, the prion protein (PrP).
ZR 101
MH Animal; Forecasting; Human; *Prion Diseases/etiology/genetics; *Prions; Species Specificity
AD Elizabeth Fisher, Glenn Telling, John Collinge, Department of Neurogenetics, Imperial College School of Medicine at St. Mary's, London, UK
SP englisch
PO USA