NR AAVK
AU Aucouturier,P.; Carp,R.I.; Carnaud,C.; Wisniewski,T.
TI Prion diseases and the immune system
QU Clinical Immunology 2000 Aug; 96(2): 79-85
PT journal article; review; review, tutorial
AB Transmissible spongiform encephalopathies are caused by unusual infectious agents that are purported to contain a single type of macromolecule, a modified host glycoprotein. The term prion has been applied to this group of agents. Surprisingly, the immune system appears to behave as a Trojan's horse rather than a protective fortification during prion infections. Because prions seem to be essentially composed of a protein, PrPsc, identical in sequence to a host encoded protein, PrPc, the specific immune system displays a natural tolerance. However, lymphoid organs are strongly implicated in the preclinical stages of the disease. Certain immunodeficient animals are resistant to prions after peripheral inoculation. In normal subjects, cells of the immune system support the replication of prions and/or allow neuroinvasion. A better understanding of these aspects of prion diseases could lead to immunomanipulation strategies aimed at preventing the spread of infectious agents to the central nervous system.
ZR 72
MH Animal; Human; Immune System/*physiology; Prion Diseases/*immunology; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Neurology, NYU Medical Center, New York, New York, USA
SP englisch
PO USA