Creutzfeldt-Jakob Disease Foundation

Prion diseases are cause by prions, which are mis-shapen forms of a normal protein in the body that become infectious and toxic to cells. Prion diseases are often difficult to diagnose because they show a wide variety of different symptoms and variable times from illness onset to death. It is thought that these variations could be due to small changes in the shape of the prions, which allows them to selectively attack some brain cells more than others. Recently we developed a new prion disease model by reprograming skin cells in the lab into small spheres of living human brain cells called cerebral organoids. New developments in organoid technology permit ‘tweaking’ of the cells in the cerebral organoid to be more like those found in different parts of the brain (i.e. the front of the brain versus the back). Using brain cells that mimic different parts of the brain, we can look at how prions attack different human brain cells and determine which cells are most vulnerable.

In this study we will combine the new human prion disease model with another relatively new technique called single-cell sequencing. The single-cell sequencing technique allows us to look at the changes occurring in every single cell in the organoid following infection with human prions. This will show us which brain cells are most affected by prion infection and what changes are happening inside them. With this information, we hope to learn what causes certain cells to be more vulnerable to infection and how we might in the future protect them from the attacking prions.

About the Researcher:

Cathryn Haigh, PhD
Laboratory of Persistent Viral Diseases (LPVD), National Institute of Allergy and Infectious Diseases (NIAID), Division of Intramural Research, Rocky Mountain Laboratories, National Institutes of Health, Hamilton, MT

Dr. Haigh began research in the prion field in 2002 and received her Ph.D. in Biochemistry from the University of Bath (UK) in May 2006. The following July, she relocated to The University of Melbourne (Australia) where she continued her work on prion diseases, initially in the Department of Pathology and later in the Department of Medicine. In 2017, Dr. Haigh accepted a tenure track position in the National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIAID, NIH) at the Rocky Mountain Laboratories where she is now the Chief of the prion biology unit.

In her 20 years of researching prions, she has studied the normal function of the prion protein, and its cleavage fragments, and the mechanisms of cell damage during disease. Her laboratory is now directed toward using stem cells to understand human prion diseases.

Recipient of:

• The Peggy J. Black Memorial Grant, contributed by Jim Black and Family
• The Katie Pohl Dopirak Memorial Research Grant, contributed by the Pohl and Dopirak Families
• The Maria D. McConnell Memorial Grant, contributed by Philip McConnell and Family
• The Tom Stivison Memorial Research Grant, contributed by Sandra (Cookie) Stivison
• The Strides for CJD Grant, contributed by the Families of the CJD Foundation