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Marcelo Barria, PhD & Alison Green, PhD

Grant Title:
Assessing the replication properties of prion protein aggregates found in archived formalin-fixed, paraffin embedded appendix tissue collected from the asymptomatic population by the highly sensitive in vitro prion amplification assay

Location:
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK

Grant Year: 2022

Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease that is related to the ingestion of meat products contaminated with prion diseases of cattle, referred as bovine spongiform encephalopathy (BSE). Despite widespread human exposure to BSE in the United Kingdom (UK), the number of vCJD cases remain low. However, recent studies examining archived appendices in the UK for the abnormal prion protein, have estimated that 1:2000 people potentially are vCJD carriers.

The aim of this study is to examine the nature of the abnormal prion protein found in these appendices to find out if it truly represents vCJD infection, using a highly sensitive prion protein amplification technique called hsPMCA. This technology amplifies small amounts of abnormal prion protein to levels which can be easily detected by routine biochemical methods. Using this technique it will be possible to identify the characteristic vCJD molecular signature. The results of this study will inform on the health protection measures required for public safety from surgical procedures and the use of blood and blood-products.

About the Researcher:

Marcelo Barria, PhD
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK

Dr Marcelo Barria is Head of the Protein Biochemistry Laboratory at the National CJD Research & Surveillance Unit (NCJDRSU), Centre for Clinical Brain Sciences (CCBS), University of Edinburgh. Dr Barria investigates the molecular pathology of prion diseases with an extensive record of publications on human prions, focusing on in vitro models.

Alison Green, PhD
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK

Dr. Alison Green is Head of CSF Diagnostics at the NCJDRSU and has extensive experience in protein aggregation assays, particularly RT-QuIC.

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