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Department of Neurodegenerative Disease
Head of Department: Professor J Collinge, BSc, MB, ChB, MD, FRCP, FRS
The Department of Neurodegenerative Disease’s research portfolio encompasses prion diseases (predominantly within the embedded MRC Prion Unit), Alzheimer’s disease and related disorders, Huntington’s disease, motor neurone disease and frontotemporal dementia and studies of the pathways of cellular senescence. The research philosophy is to combine basic and clinical translational research; many of the key contributions towards understanding the basic biology of these diseases have come from clinical and neuropathological observations, and efficient translation of basic laboratory research to the clinic is essential to provide benefits for patients at the earliest opportunity.
Dementia Research Centre
The possibility of disease modification therapies in the degenerative
dementias has accelerated the pace of research into improving early
diagnosis and monitoring of disease progression.
The Dementia Research Centre has a number of multi-disciplinary research projects with a tight clinical focus. Structural and functional brain imaging techniques are being used to identify the earliest features in familial Alzheimer’s disease and familial frontotemporal dementia cohorts. Image analysis techniques developed at the Centre have been adopted by a number of research groups worldwide to analyse multi-centre trials where rates of brain atrophy have been chosen as outcome measures. An active programme of neuropsychological research at a group level, as well as single case studies, has helped to characterise cognitive and behavioural profiles of some of these diseases as well as providing insights into the neurobiology of dementia and cognitive function more generally. The Centre has been involved in a number of important multicentre treatment trials, including ongoing studies of immunotherapy in Alzheimer’s disease. Studies in collaboration with the MRC Cyclotron Unit at the Hammersmith Hospital are producing exciting results on imaging amyloid burden in vivo. Strong and longstanding collaboration with other departments at the ION, particularly in the fields of pathology and genetics, have continued to prove very productive. The research programmes within the Centre are all very closely linked with our clinical practise and this includes work on improving care and support for patients and families affected by these devastating disorders.
For more information see the DRC website
MRC Prion Unit
The research strategy is aimed at both rapid developments to target key areas of public health concern and a long-term approach to the understanding of prion disease and the wider relevance of prion-like molecular mechanisms in the pathobiology of neurodegeneration. The Unit provides a key training resource and has developed the required infrastructure and patient base, in partnership with the NHS and MRC Clinical Trials Unit, to enable the UK’s first therapeutic trial into prion diseases to be established in 2004.
Prion diseases are also called the transmissible spongiform encephalopathies (TSEs) and they occur in humans and various animal species. The best known animal prion diseases are scrapie, a naturally occurring disease of sheep and goats; and bovine spongiform encephalopathy (BSE) of cattle. They are degenerative brain disorders and, although transmissible, they do not pass easily between humans; transmission requires exposure to infected tissue such as brain. This has occurred as a result of certain medical and surgical procedures where instruments or medicines were accidentally contaminated with prions. With variant Creutzfeldt- Jakob disease (vCJD), there is now evidence it can also be transmitted by blood transfusion. In an area of Papua New Guinea, up until the late 1950’s, an epidemic human prion disease called kuru was transmitted by exposure to infected human tissues of deceased relatives (endocannibalism). Prion diseases have long been known to be able to cross the species barrier and this has been supported by key laboratory experiments. Tragically, BSE prions appear able to infect humans and have caused the novel human prion disease, variant CJD. It was the work of the Unit staff that provided, within a few months of the recognition of variant CJD in March 1996, the first experimental evidence that variant CJD was human BSE – by demonstration that variant CJD had the molecular “signature” of BSE and was caused by the same prion strain type. This conclusion was later strengthened by animal studies published by Unit staff and other groups, and is now generally accepted in the scientific community.
For more information see the Prion Unit website