Prion Disease by James A. Mastrianni

The Role of the AGAAAAGA Palindrome in Prion Disease
2008 Seed Grant
James A. Mastrianni, M.D., Ph.D.
University of Chicago

There is a great urgency to better understand the manner in which prions (PrPSc) generate and
propagate, as BSE (mad cow disease) is currently a major health threat abroad and the rapid
spread of CWD throughout the U.S. is a major concern here at home. The BSe outbreak has
led to the development of a TSE Advisory group to the FDA, to ensure the safety of the U.S.
blood supply. In addition, the spread of CWD HAS Led to the mandatory culling of deer and elk
in several states. Thus, understanding the mechanism and determinants of the transmissible
capacity of prion disease is of great importance. Inherent within our studies is the potential to
find treatment strategies for prion disease. Our spontaneous disease mouse models will provide
insight into the genetic prion disease determinants and will eventually prove useful for the
testing of potential treatments for prion disease.

Other Grants

Rebekah C. Evans, Ph.D., Georgetown University
In Vivo and Ex Vivo Dissection of Midbrain Neuron Activity During Exercise
Exercise is important for the health of the body and the mind. Exercise promotes learning and reduces symptoms of brain-related diseases such as Parkinson’s disease and Alzheimer’s disease. However, it…
William J. Giardino, Ph.D. Stanford University
Deciphering the Neuropeptide Circuitry of Emotional Arousal in Narcolepsy
This research project aims to investigate the neural mechanisms of a specific type of brain cell called neuropeptide neurons within a region of the brain’s amygdala network called the bed…
Howard Gritton, Ph.D., University of Illinois
Attention Mechanisms Contributing to Auditory Spatial Processing.
Our world is composed of a rich mixture of sounds. We often process sounds including speech in the presence of many other competing auditory stimuli (e.g., voices in a crowded…
Nora Kory, Ph.D., Harvard University
Elucidating the Fates and Functions of Lactate in the Brain
The human brain requires significant energy to function. Despite accounting for only 2% of our body weight, the brain consumes a substantial 20% of the body’s energy, relying on a…