Prions cannot replicate and cause disease without the presence of the normal cellular form of the prion protein. A feasible way to prevent prion disease in at-risk individuals, such as those who carry a mutation associated with disease, is to reduce the expression of the prion protein. To be effective this may require long-term (lifetime) reduction of the expression of the prion protein. However, it is not known what the consequences of long-term reduction of prion protein expression is. Some normal functions of the prion protein could contribute to the development of cancer and the prion protein has been observed to increase in some cancers. This project will investigate the effect of changing prion protein expression on the risk of cancer.
Cancer occurs when damage to genes causes a cell to replicate or prevents a cell from dying. These cells form a mass that is called a tumor. Like an accelerator or brake pedal can change the speed of a car, damage to genes can change tumor development. The damage to genes, called a mutation, can be thought of as either forcing an accelerator pedal down or taking a brake off. A car with the accelerator on and brake off will increase speed quickly. There are also genes that might act as gears, able to increase or decrease cancer progression depending on how much protein is expressed. We believe that the prion protein might be acting as a gear to affect the progression of cancer.
We have observed that in some cancers reducing prion protein expression makes cancer worse. In this project we will investigate how the prion protein contributes to cancer by investigating how prion protein expression changes with development of cancer from low grade to high grade cancer and how the presence or absence of the prion protein affects tumor progression. The results of this project will establish whether patients receiving treatment to reduce the risk of prion disease through reduced expression of the prion protein require additional monitoring for cancer.
Victoria Lawson, PhD
Department of Microbiology and Immunology, Peter Doherty Institute for Infection and Immunity, The University of Melbourne, Victoria, Australia
Risk of cancer from reduced expression of PrPC-implications for prion disease treatment
Dr. Lawson received her PhD in virology from the University of Melbourne, Australia and completed post-doctoral training in prion disease at the National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIAID, NIH) Rocky Mountain Laboratories (USA) before returning to Australia as a Howard Florey Centenary Fellow in the Department of Pathology, The University of Melbourne. She is currently an Associate Professor in the discipline of pathology in The Department of Microbiology and Immunology at The University of Melbourne with research interests in infectious diseases, neurodegeneration and cancer.
Contributed by: The Families of the CJD Foundation
Funds raised by the annual Strides for CJD run/walk have been applied to research grants awarded since 2016.
Contributed by: The Families of the CJD Foundation
Funds donated by supporters of the CJD Foundation have been applied to research grants awarded since 2009.
This website was made possible by a generous donation from Cookie Stivison, in memory of her husband Tom Stivison, and a grant from the Centers for Disease Control and Prevention.
