Researchers have developed new insight into a rare but deadly brain infection, called progressive multifocal leukoencephalopathy (PML). This disease – which is caused by the JC virus – is most frequently found in people with suppressed immune systems and, until now, scientists have had no effective way to study it or test new treatments.
The JC virus is an example of an infection that specifically targets glia, the brain’s support cells,
said neurologist Steve Goldman, M.D., Ph.D., co-director of University of Rochester Center for Translational Neuromedicine and senior author of the paper. Because this virus only infects human glia and not brain cells in other species, it has eluded our efforts to better understand this disease. To get around this problem, we have developed a new mouse model that allows us to study human glia in live animals.
The new discovery – which appears today in the Journal of Clinical Investigation – was the result of research using a new tool developed at the University of Rochester. Last year, Goldman and Maiken Nedergaard, M.D., D.M.Sc., reported that they had created a mouse model whose brains consisted of both animal neurons and human glia cells. While the previous study focused on the fact that the human cells essentially made the mice smarter, at the same time it created a powerful new platform for researchers to study human glial cells in live adult animals, including diseases that impact these cells.