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.
It 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. (...)
Mots-clés Pascal : Encéphalopathie spongiforme Creutzfeldt Jakob, Infection, Prion, Transmission, Boeuf, Artiodactyla, Ungulata, Mammalia, Vertebrata, Epidémiologie, Surveillance sanitaire, Recommandation, Homme, Article synthèse, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Maladie dégénérative
Mots-clés Pascal anglais : Creutzfeldt Jakob disease, Infection, Prion, Transmission, Ox, Artiodactyla, Ungulata, Mammalia, Vertebrata, Epidemiology, Sanitary surveillance, Recommendation, Human, Review, Nervous system diseases, Central nervous system disease, Cerebral disorder, Degenerative disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0176269
Code Inist : 002B17G. Création : 16/11/1999.