The ALS Association

ALS Ice Bucket Challenge Progress

New Disease Model Highlights Astrocyte Contribution to ALS

February 19, 2015

A significant new model of ALS has been created that will help elucidate the contribution of cells called astrocytes to the disease. Astrocytes are support cells for neurons within the central nervous system and have been previously implicated in the ALS disease process.

Researchers led by Association-funded researcher Su-Chun Zhang, M.D., Ph.D., of the University of Wisconsin at Madison, implanted cells called neural progenitors into the spinal cords of adult mice. The neural progenitors were derived from either human embryonic stem cells or induced pluripotent stem cells (iPS cells), which are derived from human skin tissue. Consistent with previous studies of transplanted neural progenitors, the cells developed into astrocytes and migrated away from the site of implantation. Over time, the astrocytes replaced the mouse’s own astrocytes, integrating into the nervous system without apparent untoward effect.

When the implanted neural progenitors were derived from people with ALS, the astrocytes also migrated and integrated into the mouse nervous system but caused motor deficits similar to those seen in genetically created ALS mouse models.

“This new model should allow us to rapidly explore the unique contribution of astrocytes to the ALS disease process,” commented Lucie Bruijn, Ph.D., MBA, Chief Scientist for The ALS Association. “We may also be able to test astrocyte-related therapies in this model, including delivery of growth factors or other molecules supplied by the astrocytes themselves. The discovery that astrocytes derived from people with ALS resulted in motor neuron deficits in these mouse models is significant and should be carefully considered as we move to developing approaches using a patient’s own cells for transplantation.”

The study was published in the Journal of Clinical Investigation.

Click here to read the press release.

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