New funding for prion disease research

Dr Mark Head and colleagues have been awarded an NC3Rs grant to develop a stem cell-derived neuronal and glial model of human prion disease.

In addition to its CJD surveillance function, the National Creutzfeldt-Jakob Disease Research and Surveillance Unit (NCJDRSU) performs basic and applied research in human prion diseases.

Coinciding with the further integration of the NCJDRSU into the Centre for Clinical Brain Sciences, and in continuing collaboration with the Roslin Institute, scientists have been successful in obtaining funding from the National Centre for Reduction, Refinement and Replacement of Animals in Research (NC3Rs) to develop a cell culture model of human prion disease. The award of £268,680 to Dr Mark Head (Principal Investigator) and co-investigators Professor Siddharthan Chandran, Professor James Ironside and Professor Jean Manson, is for a two-year study to use induced pluripotent stem cell-derived neurons and astrocytes to establish the first fully in vitro cell culture model of human prion infection and replication in human cells.

Prion disease research benefits from excellent animal models and cell-free prion protein conversion model systems, but there is a manifest gap in the area of cell biology. The absence of such a cell culture model in which human prions replicate in human cells has hampered prion disease research. A stem cell derived model system will support mechanistic studies of human prion infection and replication, and will facilitate drug discovery for these uniformly fatal diseases. This is only the second prion disease project funded by NC3Rs and only the eleventh NC3Rs award to the University of Edinburgh in any field of study. The award will involve a conference on strategies for the replacement, refinement and reduction of animals in neurodegeneration research, the details of which will follow.