The NCJDSU Tissue Resource: support for continued banking activities

Prion diseases are fatal disorders that affect the brain, and include conditions such as BSE or ?mad cow disease? in cattle and Creutzfeldt-Jakob disease (CJD) in humans. One form of CJD, known as variant CJD, is linked to exposure to BSE through the diet. Prion diseases are transmissible, and studies of CJD and BSE have shown that the infectious agent accumulates at highest levels in the brain. However, the agent can also be present in a range of other tissues throughout the body. The aim of this research is to continue the collection and storage of tissues from all forms of CJD in a brain and tissue bank in the National CJD Surveillance Unit, Edinburgh. This bank played a key role in the discovery of variant CJD, and is in continuing use by a wide range of researchers in UK and across the world. The material collected comes from tissue and organs taken at autopsy from patients with suspected CJD only when the relatives have given consent. We also collect blood and CSF samples from living patients, again with consent. The bank has approval for this work from our local Ethical Committee, and has been used for many research projects, resulting in major scientific and medical publications. The information from this research has also made a big impact on the work of the NHS, particularly in steps to reduce the chance of transmission of variant CJD by blood transfusion and by surgical instruments. We believe that the bank will continue to be needed by researchers in the UK and elsewhere in the future, since there are so many unanswered questions about this group of diseases. There is also a major need to help develop a test for CJD that can be used on living patients, which will be helped by the use of the tissue and blood samples in our bank. We run the bank according to MRC guidelines, and use a Steering Committee (that includes 2 members of the public) to oversee our work. The results from our work are presented in many forms, such as scientific and medical papers, lectures (including public lectures) and in interviews with the press, radio and television.

This application seeks to renew MRC support for the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) tissue resource (part of the MRC Edinburgh Brain Banks), in order to address research needs in the UK and overseas. Creutzfeldt-Jakob disease (CJD) is one of a group of fatal transmissible neurodegenerative disorders known as prion diseases. These disorders have reached enormous significance in UK since the epidemic of bovine spongiform encephalopathy (BSE) and the subsequent emergence of variant CJD (vCJD), which results from BSE infection in humans. The identification and characterisation of vCJD was critically dependent on the availability of a wide range of tissues, blood, cerebrospinal fluid and DNA and in a variety of forms (fixed, frozen etc) from the tissue resource in the NCJDSU. This resource is recognised as a unique collection and has been used by all the leading research groups in the field on a worldwide basis. The continuing uncertainties over the incidence of vCJD infection in the UK population, the tissue distribution of infectivity in all forms of human prion disease, the need to study the strains of the infectious agent in human prion disease and to understand the mechanisms of disease pathogenesis all reinforce the requirement for well characterised tissues that are available in a range of forms to maximise potential research use. The demands for materials in the NCJDSU tissue resource are not confined to vCJD, and encompass all forms of human prion disease (except kuru). The support requested is modest and comprises one technical post, support for two new freezer and consumables to cover running costs. The infrastructure of NCJDSU (funded by the Department of Health) allows a range of backup facilities (e.g. computer support, staff cover, tissue processing facilities) that allow this to be a cost-effective application. The NCJDSU tissue resource has LREC approval to store and use autopsy tissues for research purposes, and operates with governance, strategic oversight and advice on resource allocation from a Steering Committee on which MRC is represented. Data arising from research performed materials from the NCJDSU resource has generated a large quantity of publications in leading scientific and medical journals, and has provided considerable input to policy formulation and risk assessment in the UK and overseas. These important activities will be not only maintained, but strengthened by this application.