Working with mouse, fly and human cells and tissue, Johns Hopkins researchers report new evidence that disruptions in the movement of cellular materials in and out of a cell’s control center – the nucleus – appear to be a direct cause of brain cell death in Huntington’s disease, an inherited adult neurodegenerative disorder.

Moreover, they suggest, laboratory experiments with drugs designed to clear up these cellular “traffic jams” restored normal transport in and out of the nucleus and saved the cells.

In the featured article published online on April 5 in the journal Neuron, the researchers also conclude that potential treatments targeting the transport disruptions they identified in Huntington’s disease neurons may also work for other neurodegenerative diseases, such as ALS and forms of dementia.

“We’re trying to get at the heart of the mechanism behind neurodegenerative diseases and with this research believe we’ve found one that seems to be commonly disrupted in many of them, suggesting that similar drugs may work for some or all of these disorders,” says Jeffrey Rothstein, MD, PhD, a professor of neurology and neuroscience, and director of the Brain Science Institute and the Robert Packard Center for ALS Research at the Johns Hopkins University School of Medicine.