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From: TSS ()
Subject: Molecular neurology of prion disease
Date: June 18, 2005 at 7:40 pm PST

Journal of Neurology Neurosurgery and Psychiatry 2005;76:906-919
© 2005 BMJ Publishing Group Ltd



Molecular neurology of prion disease
J Collinge

Correspondence to:
J Collinge
MRC Prion Unit and National Prion Clinic, Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK;

Prions are infectious pathogens principally composed of abnormal forms of a protein encoded in the host genome. They cause lethal neurodegenerative conditions including CJD, GSS, and kuru in humans and scrapie and bovine spongiform encephalopathy in domestic animals. Remarkably, distinct strains of prions occur despite absence of an agent-specific genome: misfolded proteins themselves may encode strain diversity – with wide implications in biology. The arrival of variant CJD, and the experimental confirmation that it is caused by infection with BSE-like prions, has focussed research on early diagnosis and treatment. Recent advances lead to considerable optimism that effective human therapies may now be developed. While several drugs have been tried in small numbers of patients, there is no clear evidence of efficacy of any agent and controlled clinical trials are urgently needed. Importantly, there is increasing recognition that fundamental processes involved in prion propagation – seeded aggregation of misfolded host proteins – are of far wider significance, not least in understanding the commoner neurodegenerative diseases that pose such a major and increasing challenge for healthcare in an ageing population.


Abbreviations: CJD, Creutzfeldt-Jakob disease; CNS, central nervous system; FDC, follicular dendritic cell; GPI, glycosylphosphatidylinositol; GSS, Gerstmann-Sträussler-Scheinker disease; LRS, lymphoreticular system; vCJD, variant CJD

Keywords: Creutzfeldt-Jakob disease; Gerstmann-Sträussler-Scheinker disease; kuru; prion; variant Creutzfeldt-Jakob disease


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