Discovery Could Aid in Development of Treatments for Fatal Brain Disease

By Cohn, Meredith; Sun, Baltimore | St Louis Post-Dispatch (MO), April 9, 2017 | Go to article overview

Discovery Could Aid in Development of Treatments for Fatal Brain Disease


Cohn, Meredith, Sun, Baltimore, St Louis Post-Dispatch (MO)


BALTIMORE * Huntington's disease is an inherited brain disorder that is uniformly fatal, but researchers at Johns Hopkins believe they have made a big discovery about how the disease progresses that could lead to a way to stop it.

Their findings offer hope for a treatment to more than 30,000 Americans who have Huntington's symptoms and 200,000 more at risk of inheriting the disease. They also could help scientists better understand other fatal brain diseases such as amyotrophic lateral sclerosis and a certain type of dementia, as well as help researchers learn more about normal brain aging.

"We found these kind of traffic jams in cells, and if we can fix the traffic jams we can potentially provide a new avenue for treatment of neurodegenerative disease," said Jonathan Grima, a graduate student in the Johns Hopkins University School of Medicine department of neuroscience whose research was published in the journal Neuron.

Grima said it's not clear whether a drug developed to clear these jams could stop cells from dying, and thus stop progression of Huntington's, but that is the goal. Grima works in the lab of Dr. Jeffrey Rothstein, director of Hopkins' Brain Science Institute. Rothstein, who normally focuses on ALS, also known as Lou Gehrig's disease, has pursued similar cell research for that disease, and a drug that may offer some treatment is being investigated.

Components of a brain cell, such as salts and proteins, need to move in and out of a cell's operations center, the nucleus, to keep it functioning properly. In people with Huntington's disease, proteins produced by the Huntington's-related gene clump together in the nucleus and can't pass through special passageways called nuclear pores every cell has many of these pores causing the cells to shut down and die.

While scientists have known there was a Huntington's gene and knew about nuclear pores, they hadn't been closely studied in the brain. They didn't know about this breakdown in so-called nuclear transport in Huntington's sufferers, Rothstein said.

The discovery was met with intense interest by Huntington's researchers. "This is very exciting research because we didn't know what mutant genes or proteins were doing in the body, and this points to new areas to target research," said George Yohrling, senior director of mission and scientific affairs at the Huntington's Disease Society of America. …

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