Prion protein-specific antibodies for therapeutic intervention of transmissible spongiform encephalopathies.
Prion diseases, also called transmissible spongiform encephalopathies, are a group of fatal neurodegenerative conditions that affect humans and a wide variety of animals. There is no therapeutic or prophylactic approach against prion diseases available at present. The causative infectious agent is the prion, also termed PrPSc, which is a pathological conformer of the cellular prion protein PrPC. Passive immunisation studies with PrPC-specific antibodies indicated that immunotherapeutic strategies directed against PrPC can prevent prion disease. In this review, putative mechanisms of antibody-mediated prion inactivation, as well as active immunisation strategies, are discussed. Special attention is given to the problem of immunological self-tolerance against PrP.
Buchholz
Christian J
CJ
Division of Medical Biotechnology, Paul-Ehrlich-Institut, Paul-Ehrlich-Str. 51-59, 63225 Langen, Germany. bucch@pei.de
Bach
Patricia
P
Nikles
Daphne
D
Kalinke
Ulrich
U
D000818:Animals; D000906:Antibodies / Q000008:administration & dosage*; D000918:Antibody Specificity; D006801:Humans; D007114:Immunization / Q000379:methods / Q000639:trends; D018621:PrPC Proteins / Q000276:immunology* / Q000378:metabolism; D017096:Prion Diseases / Q000276:immunology* / Q000517:prevention & control*
D000906:Antibodies; D018621:PrPC Proteins