Brain Advance Access originally published online on July 1, 2006
Brain 2006 129(9):2241-2265; doi:10.1093/brain/awl150
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Review Article |
A systematic review of prion therapeutics in experimental models
MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London Queen Square, London, UK
Correspondence to: John Collinge, MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK E-mail: j.collinge{at}prion.ucl.ac.uk
Prion diseases are transmissible, invariably fatal, neurodegenerative diseases which include Creutzfeldt–Jakob disease (CJD) in humans and bovine spongiform encephalopathy and scrapie in animals. A large number of putative treatments have been studied in experimental models over the past 30 years, with at best modest disease-modifying effects. The arrival of variant CJD in the UK in the 1990s has intensified the search for effective therapeutic agents, using an increasing number of animal, cellular and in vitro models with some recent promising proof of principle studies. Here, for the first time, we present a comprehensive systematic, rather than selective, review of published data on experimental approaches to prion therapeutics to provide a scientific resource for informing future therapeutics research, both in laboratory models and in clinical studies.
Key Words: prion; Creutzfeldt–Jakob disease; transmissible spongiform encephalopathy; experimental models; therapeutics
Abbreviations: CJD, Creutzfeldt–Jakob disease; CR, congo red; dpi, days post infection; DS, dextran sulphate; GAG, glycosaminoglycans; HM, heparan mimicking; HPA-23, heteropolyanion-23; IC50, half-maximal inhibition concentration in vitro; i.c., intracerebral; i.c.v., intracerebralventricle; i.p., intraperitoneal; iv, intravenous; LRS, lymphoreticular system; PPS, pentosan polysulphate; PrP, prion protein; PrPC, cellular prion protein; PrPSc, disease-associated prion protein; PrPsen, protease-sensitive prion protein; PrPres, protease-resistant prion protein (as monitored by proteinase-K resistant PrP immunoreactivity); Prnp, prion protein gene; SAF, scrapie-associated fibril; sc, subcutaneous (peripheral)
Received January 6, 2006. Revised May 4, 2006. Accepted May 8, 2006.
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