Brains of mice with human glial cells containing Huntington's disease mutation (top image) show significantly less myelin (red) than controls (bottom image).

New research gives scientists a clearer picture of what is happening in the brains of people with Huntington's disease and lays out a potential path for treatment. The study, which appears today in the journal Cell Stem Cell, shows that support cells in the brain are key contributors to the disease.

"Huntington's is a complex disease that is characterized by the loss of multiple cell populations in the brain," said neurologist Steve Goldman, M.D., Ph.D., the lead author of the study and the co-director of the Center for Translational Neuromedicine at the University of Rochester Medical Center (URMC). "These new findings help pinpoint how the genetic flaw in Huntington's gives rise to glial cell dysfunction, which impairs the development and role of these cells, and ultimately the survival of neurons. While it has long been known that neuronal loss is responsible for the progressive behavioral, cognitive, and motor deterioration of the disease, these findings suggest that it's glial dysfunction which is actually driving much of this process."

Huntington's is a hereditary and fatal neurodegenerative disease characterized by the loss of medium spiny neurons, a nerve cell in the brain that plays a critical role in motor control. As the disease progresses over time and more of these cells die, the result is involuntary movements, problems with coordination, and cognitive decline, depression, and often psychosis. There is currently no way to slow or modify the progression of this disease.