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MRC Prion Unit at UCL, Institute of Prion Diseases

Update on PRN100: an anti-prion protein monoclonal antibody developed by the MRC Prion Unit for treatment of human prion diseases including Creutzfeldt-Jakob disease (CJD)

University College London Hospitals NHS Foundation Trust (UCLH) will be treating a patient with the antibody after the Court of Protection confirmed on 8th October 2018 that it is lawful and in the patient's best interests to receive the treatment.  A statement and Q&A giving more details are available on the UCLH website at http://www.uclh.nhs.uk/News/Pages/PrionCJD.aspx

We pursue a long-term research strategy with a mission to develop diagnostic tests and treatments for these incurable disorders through an understanding of their fundamental biology.

In addition to its research mission, the Institute of Prion Diseases is dedicated to training skilled researchers, committed to advancing and disseminating knowledge and technology to improve the quality of life and economic competitiveness of the UK and promote dialogue with the public, politicians, the media and other stakeholders about medical research.

The Institute is closely linked to the NHS National Prion Clinic (NPC) - a national referral centre for prion diseases, based at the National Hospital for Neurology and Neurosurgery (part of UCLH NHS Foundation Trust). The partnership and integration that exists between the Institute and NPC provides a unique environment for training, developing treatments and expertise in this area of medicine as well as supporting neurodegenerative disease research more widely.

What are prion diseases?

Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a group of progressive neurodegenerative conditions.

The most well-known prion disease is bovine spongiform encephalopathy (BSE), which exists in cattle, and the human variant which was first described by Creutzfeldt and Jakob and thus became known as CJD (Creutzfeldt-Jakob disease).

Prions, unlike other infectious agents or germs, appear to lack their own genes and consist of aggregated misshapen forms of one of the body’s own proteins, the prion protein or PrP. These rogue proteins tend to accumulate in the brain (and some other organs) causing damage, which interferes with normal brain function. These diseases in humans are now grouped together according to whether they are sporadic, inherited, or acquired.