Prion Disease

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

Lindsay M. De Biase, Ph.D., University of California Los Angeles
The Role of Microglial Lysosomes in Selective Neuronal Vulnerability
Synapses, the sites of signaling between neurons in the brain, play essential roles in learning, memory, and the health of neurons themselves. An enduring mystery is why some neurons are…
How the Nervous System Constructs Internal Models of the External World
As animals navigate their environments, they construct internal models of the external sensory world and use these models to guide their behavior. This ability to incorporate ongoing sensory stimuli into…
Xiaojing Gao, Ph.D., Stanford University
When Neural Circuits Meet Molecular Circuits: Quantitative Genetic Manipulation with Single-cell Consistency
Cells are the building blocks of our bodies. We get sick when the cells “misbehave”. The way modern gene therapies work is to introduce genes, fragments of DNA molecules that…
Rafiq Huda, Ph.D., Rutgers University
Conducting the Orchestra of Movement—Functional Role of Striatal Astrocytes in Health and Disease
Movement requires coordinated activity across a large brain-wide network. The striatum is a particularly important part of this circuit; it integrates motor-related information from many distinct brain regions to regulate…