Approaches to therapy of prion diseases.
Devising approaches to the therapy of transmissible spongiform encephalopathies, or prion diseases, is beset by many difficulties. For one, the nature of the infectious agent, the prion, is understood only in outline, and its composition, structure, and mode of replication are still shrouded in mystery. In addition, the mechanism of pathogenesis is not well understood. Because clinical disease affects mainly the brain parenchyme, therapeutic agents must be able to traverse the brain-blood barrier (BBB) or have to be introduced directly into the cerebrospinal fluid or brain tissue. And finally, because the disease is usually recognized only after onset of severe clinical symptoms, the question arises as to whether the neurodegenerative processes can be reversed to any extent after a successful eradication of the agent.
Weissmann
Charles
C
Department of Neurodegenerative Disease/MRC Prion Unit, Institute of Neurology, Queen Square, London WC1N 3BG, UK. charles.weissmann@prion.ucl.ac.uk
Aguzzi
Adriano
A
D000818:Animals; D000890:Anti-Infective Agents / Q000008:administration & dosage* / Q000009:adverse effects; D017209:Apoptosis / Q000187:drug effects; D001927:Brain Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology; D002417:Cattle; D045744:Cell Line, Tumor; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology / Q000635:transmission; D003430:Cross-Sectional Studies; D005786:Gene Expression Regulation / Q000502:physiology; D006801:Humans; D007276:Injections, Intraventricular; D009447:Neuroblastoma; D019636:Neurodegenerative Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology; D009474:Neurons / Q000187:drug effects; D017096:Prion Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology / Q000635:transmission; D011328:Prions / Q000235:genetics / Q000472:pathogenicity
D000890:Anti-Infective Agents; D011328:Prions