Creutzfeldt Jakob Disease Clinical Research Group

Creutzfeldt-Jakob disease (CJD) is one of the human forms of the transmissible spongiform encephalopathies (TSE), also referred to as prion diseases.

Human prion diseases are a rare group of fatal neurodegenerative diseases, which have currently no cure. In most cases, they are marked by a rapid progression of symptoms. Clinical features of prion diseases comprise varying combinations of neurological signs and symptoms including dementia, psychiatric symptoms, incoordination of movements (ataxia, dysarthria), myoclonus (muscle jerks), weakness, spasticity, chorea, seizures, and/or autonomic disturbances. These diseases affect approximately 1 to 2 per people in every one million worldwide per year. Prion diseases can affect humans and animals, the best know prion disease in animals is bovine spongiform encephalopathy (BSE). Prion diseases are unique in that they are transmissible and can be genetic.

The laboratory is currently focused on the refinement and utilisation of our recently developed, novel in vitro and in vivo acute prion neurotoxicity models to better understand pathogenesis with a view to developing targeted treatments. The in vitro model is based on an electrophysiology paradigm harnessing a multi-electrode array platform and murine hippocampal slices while the in vivo model employs a battery of behavioural testing shortly after precise stereotaxic delivery of prions to the hippocampal CA1 region of mice.

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