Dr. Barney Viengkhou is a postdoctoral fellow and PhD graduate whose research involves studying the cellular mechanisms involved in interferon-alpha mediated brain diseases. During his PhD, he has identified that endothelial cells are driving pathophysiology in mice with chronic overproduction of interferon-alpha in the brain, which forms the basis of his application.
Are the changes to brain vasculature in Aicardi-Goutières syndrome mirrored in mice with chronic interferon-alpha production in the brain?
PI: Barney Viengkhou
ABSTRACT/SUMMARY OF PROJECT
The genetic basis of Aicardi-Goutières syndrome (AGS) involves several genes. Although several animal models replicate these genetic changes, they all have largely failed to mirror the symptomology and neuropathology observed in patients. Despite the number of genes associated with AGS, a common feature in patients is the presence of chronically elevated interferon- in the brain. Importantly, transgenic mice that chronically overproduce interferon- in the brain mirror the disease and brain pathology observed in patients with AGS. We previously identified that the brain vasculature is primarily mediating the pathology in the transgenic mice. This project will translate the mouse findings to the human disease by characterizing and investigating the morphological and molecular changes in the brain vasculature. Currently, there is no cure for AGS and treatments largely manage symptoms. Hence, demonstrating that the blood vessels of the brain are similarly affected in mice and humans is critical for understanding the cellular mechanisms of disease but also enables these mice to be utilized to develop and test new treatment strategies for AGS.