The ALS Association

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Carrie Munk
The ALS Association
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Nervous System Support Cells are Toxic to Motor Neurons in Sporadic ALS Model

Washington, D.C. (February 21, 2014)—In work supported by The ALS Association, scientists have discovered new insights into the causes of motor neuron death in ALS. The findings are likely to lead to testing of new therapeutic strategies to slow the progression of the disease. The study was published in the journal Neuron.

ALS (amyotrophic lateral sclerosis), otherwise known as Lou Gehrig’s Disease, is a progressive neurodegenerative disease that affects neurons (nerve cells) in the brain and the spinal cord. Eventually, people with ALS lose the ability to initiate and control muscle movement, which often leads to total paralysis and death within two to five years of diagnosis. There is no cure and no life-prolonging treatments for the disease. 

Previous work has shown that an unknown toxic property of supporting cells, called astrocytes, contributes to the death of motor neurons in ALS. Much of that work has been done in a disease model with artificially elevated levels of the ALS-causing protein, superoxide dismutase (SOD). To test whether a similar mechanism was at work without that protein elevation, researchers grew astrocytes from ALS patients with the sporadic (non-familial) form of the disease and exposed them to human motor neurons from healthy embryonic stem cells. This is one of the first times that such a “fully humanized” model of sporadic ALS combining astrocytes and motor neurons has been produced.

The researchers found that motor neurons succumbed to the toxic effects of the astrocytes, through a specific process of cell death called necroptosis. Because this process is at least partially understood on the molecular level, it should be possible to design treatments to interrupt it, which may be therapeutic.

The research was performed by Diane Re, Ph.D., and Virginia Le Verche, Ph.D., under the leadership of Serge Przedborski, M.D., Ph.D., all of Columbia University in New York and was funded by The ALS Association, Greater New York Chapter.

“These exciting results shed new light on the interaction between motor neurons and astrocytes,” said Lucie Bruijn, Ph.D., Chief Scientist for The Association. “The finding that astrocytes from sporadic ALS patients exert toxicity will strengthen the focus on understanding this interaction more closely and point out possible new targets for therapy.”

About The ALS Association
The ALS Association is the only national non-profit organization fighting Lou Gehrig’s Disease on every front.  By leading the way in global research, providing assistance for people with ALS through a nationwide network of chapters, coordinating multidisciplinary care through certified clinical care centers, and fostering government partnerships, The Association builds hope and enhances quality of life while aggressively searching for new treatments and a cure.  For more information about The ALS Association, visit our website at

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