The Roslin Institute TSE Resource Centre

The Transmissible spongiform encephalopathy (TSE) diseases (also known as Prion diseases) are fatal neurodegenerative diseases of animals and humans. These include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and Creutzfeldt-Jakob disease (CJD) in humans. TSEs are infectious diseases and can be transmitted within and between species. It is thought that the transmission of BSE to humans via contaminated foodstuffs resulted in the emergence of variant CJD (vCJD), which to date has caused 164 deaths in the UK. Four of these cases have occurred following a blood transfusion from an individual who later went on to develop vCJD, indicating that human to human transmission of vCJD can occur, and the potential exists for further human to human spread via surgery, dentistry and other invasive procedures. Although the incidence of BSE and vCJD appear to be declining, new TSEs of animals are constantly being identified, such as atypical scrapie, atypical forms of BSE (H-type and L-type), and chronic wasting disease (CWD) in North American deer and elk. The risks posed to humans from these infectious agents are unknown. For these reasons, continued research into the causes of disease and mechanisms of neurodegeneration are essential. Only by identifying the nature of the TSE infectious agent and how it destroys neurons will effective therapies and pre-mortem diagnostic assays be developed. In order to perform such research, specialised reagents are required. TSE agent strains can only be truly propagated in animals, and such reagents can only be generated or processed in laboratories with specialised facilities. In the UK, three resource centres supply specialised materials for TSE research; the CJD Resource Centre at NIBSC holds tissue from human cases of disease, the TSE Archive at VLA holds an extensive archive of ovine and bovine TSE infected tissues, and the TSE Resource Centre at the Roslin Institute supplies experimental mouse TSE agents, antibodies and cell lines. The provision of material from all three TSE archives is important to sustain TSE research in the UK. In this proposal to The BBR Fund, we are seeking support for the Roslin Institute TSE Resource Centre that will allow its development into a self sustaining resource with improved catalogue and on-line user access. This development will safeguard the future of the TSE Resource Centre and allow the continued supply of high quality reagents produced as a result of the world class TSE research performed by researchers in the Roslin Institute-Neuropathogenesis Division, to the TSE research community.

TSE (or Prion) diseases are fatal infectious neurodegenerative diseases of humans and animals. Despite many years of research, the true nature of the TSE infectious agent remains unknown, and the mechanisms by which TSE infection leads to neurodegeneration and death are undetermined. No therapy exists for treatment of TSE disease, and we have yet to produce an effective, sensitive and specific pre-mortem blood test which can identify individuals in the pre-clinical stages of disease. Although BSE and vCJD appear to be declining in the UK and Europe, cases of BSE born after the reinforced feed ban are still being identified, and human to human transmission of vCJD has occurred following blood transfusion from preclinical cases of vCJD. Additionally, new TSEs of animals such as atypical scrapie, H-type and L-type BSE, and chronic wasting disease are still being identified, and the risks posed to humans from these agents are unknown. TSEs are therefore still important diseases that pose significant public health and animal health risks. Continued research is required to characterise the nature of the agent and its mechanisms of neurodegeneration, and also to develop therapeutics and pre-mortem diagnostic assays. The Neuropathogenesis Division of the Roslin Institute is a world class centre of TSE research. Many reagents produced as part of this research are made available through the TSE Resource Centre, which in association with the NIBSC CJD Resource Centre and the VLA TSE archive provide a wide range of TSE agents, models and reagents which are required to support TSE research in the UK and worldwide. We aim to develop the TSE-RC into a self sustaining resource providing an increased catalogue of reagents at realistic cost to the research community. This application to the BBR Fund is to support the transition of the resource following its relocation to Edinburgh and secure its future in supplying reagents for TSE research.