APH 1998, 56, 169-186:

Transmissible spongiform encephalopathies of prion protein diseases and public health.

D. De Bleser, and J. Plum

Keywords: BSE, Creutzfeldt-Jakob disease, prion, NvCJD, scrapie

Prion diseases are transmissible neurodegenerative disorders of humans and animals. The prion protein (PrPc) gene is expressed to some extent in many cell types but principally in neurons. Normal PrPc may contribute in the protection of neurons and is protease sensitive. Abnormal prions consist of a post-translationally modified form of PrP, PrPsc, which is partly protease resistant. PrPsc is a protein with high resistance to inactivation by irradiation, heat and harsh chemical treatments. lt is currently proposed that PrPsc is an infectious protein that propagates by inducing the normal PrPc to become the abnormal PrPsc. PrPsc causes transmissible spongiform encephalopathies (TSE), an unusual group of degenerative brain diseases that can be transmitted by inoculation or ingestion of diseased brain or other tissues. The human diseases occur in an inherited, acquired and sporadic form. Transmission of prion diseases between species is limited by a species barrier, dertermined in part by the degree of sequence homology between the host PrP and inoculated PrPsc. The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom is a new disease that has affected over 160,000 cattle and has presumably arisen from dietary exposure to PrPsc from sheep with scrapie. Until shown otherwise we must assume that oral consumption of infectious BSE protein is a new factor for Creutzfeldt-Jakob (CJD) disease in man. Early diagnostic markers for the disease to facilitate the diagnosis and to screen blood and organ donors are not available. The control of the disease relies on measures to eradicate the spread of BSE by banning the use of ruminant tissues in ruminant feed and slaughter and rendering procedures to ensure inactivation of prions of alf infected animals. The control of nvCJD is based on reduction of exposure to BSE by banning a variety of tissues for consumption. A surveillance worldwide is increased for both BSE and nv-CJD and the WHO update regularly recommendations to limit the risk of transmitting the disease.