Broken Cellular Communication Contributes to Huntington’s Disease Symptoms

Researchers from Indiana University recently found that broken communication in a specific region of the brain plays a role in the involuntary physical movements that affect individuals with Huntington’s disease. The study suggests a “cell-interaction model” of Huntington’s disease, in which this genetic mutation causes erroneous communication between the brain’s cerebral cortex and the striatum — a portion of the forebrain most affected by the disease. To conduct their study, researchers used three mouse-models: one that had the mutant huntingtin gene in the striatum (but suppressed in the cerebral cortex), a second with Huntington’s disease where the mutant huntingtin gene was not suppressed and a third healthy control group. To assess function and communication between brain regions, scientists measured the electrical firing patterns in the cortex and striatum of the three types of mice as they moved freely. The differences in electrical activity were observable in behavior. The mice that had the mutant huntingtin gene suppressed in the cerebral cortex exhibited fewer motor control problems compared to the other mice with Huntington’s disease. “These results strongly indicate that mutant huntingtin gene in the brain’s cortical outputs is a key contributor to the physical symptoms observed in people with Huntington’s disease,” said the study’s lead researcher. The partial, but significant, improvement in both striatal neuronal activity and behavior in mice that had the mutant huntingtin gene suppressed in the cerebral cortex suggest that striatal interactions with the cortex play a key role in the motor dysfunctions of Huntington’s disease. To read more about this study, click here.