Study Funded by The ALS Association Finds Disease Worsened by Spinal Cord Cells That Normally Support Neurons

Apr 02, 2013, 08:55 ET from The ALS Association

WASHINGTON, April 2, 2013 /PRNewswire-USNewswire/ -- In work supported by The ALS Association and funded through its Milton Safenowitz Post-Doctoral Fellowship Program, researchers in Baltimore, Md., have shown that cells that normally support neurons in the spinal cord instead worsen the disease in an animal model of amyotrophic lateral sclerosis (ALS). The study was published in the journal Nature Neuroscience on March 31.

ALS, also known as Lou Gehrig's Disease, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. The disease robs people of the ability to walk, to talk and even blink an eye. It traps them inside a body they no longer can control and ultimately prevents them from breathing as it takes their life.  There is no known cause of the disease, although military veterans are approximately twice as likely to develop ALS as the general population.

In the study, researchers examined spinal cord cells called oligodendrocytes. These cells form the layer of myelin that wraps around and insulates neurons, including motor neurons. They also provide metabolic support to neurons by supplying nutrients. The death of motor neurons is the final cause of ALS.

The researchers found that in a mouse model of ALS, caused by mutations in the SOD1 gene, oligodendrocytes degenerated before the mice displayed symptoms of the disease. When the mutant gene was removed from the oligodendrocytes, disease onset was delayed, and disease severity was reduced, even though the gene remained active in other cell types, including motor neurons. Oligodendrocyte dysfunction was also seen in both the brain and spinal cord of people with ALS.

"These results strengthen the case that the ALS disease process affects other types of cells besides motor neurons," said Lucie Bruijn, Ph.D., Chief Scientist for The Association. "This will allow us to look for therapies affecting not just motor neurons but also oligodendrocytes. Targeting these cells may offer us a chance to support motor neurons before they are lost."

That strategy may be aided by current work in the field of multiple sclerosis (MS), in which oligodendrocytes are also involved. Replacing oligodendrocytes using stem cells may also be a potentially viable strategy based on research in spinal cord injury.

The research was performed by Shin Kang, Ph.D., and Ling Yi, Ph.D., both of Johns Hopkins University in Baltimore, Maryland, under the guidance of Jeffrey Rothstein, M.D., Ph.D., and Dwight Bergles, Ph.D., also of John Hopkins University.

Dr. Li received The Milton-Safenowitz Post-Doctoral Fellowship from The ALS Association in 2011. She is currently a post-doctoral fellow in Dr. Rothstein's lab. The Milton Safenowitz Post-Doctoral Fellowship for ALS Research Award encourages and facilitates promising young scientists to enter the ALS field. Funding for this two-year research award is made possible by the generosity of the Safenowitz family through the Greater New York Chapter of The ALS Association and is in memory of Mr. Safenowitz, who died of ALS in 1998.

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

SOURCE The ALS Association