astrocyte als neuroinflammation neuroprotection stem cell


ALS Detox? Healthy astrocytes may help keep motor neurons alive in people with ALS by detoxifying the nervous system. Image: Andrew Swift for Duke University School of Medicine.

More than 50% of motor neurons are lost by some estimates in people with ALS before the first signs of disease. To turn the tide of destruction, clinicians hope to keep existing motor neurons healthy in their patients by increasing supplies of neuroprotective substances.  But whether these strategies are helpful to people with ALS remains hotly debated.

A growing number of neurologists suspect that the introduction of healthy astrocytes might provide ‘life support’  to existing motor neurons in people with ALS.  And, protect them from destruction.

The stem cell-based strategy, known as astrocyte replacement, involves the injection of astrocyte precursors directly into the spinal cord. The transplantation-based approach according to preclinical studies may work in part by reducing the build up of potentially toxic levels of glutamate in the brain and spinal cord.

One of these potential treatments, known as Q cells, is approaching the clinic. The strategy, developed by Johns Hopkins University School of Medicine’s Nicholas Maragakis MD, is emerging as one of a growing number of potential personalized medicines that may benefit people with certain forms of sporadic and inherited disease.

At the 2013 Northeast ALS Consortium Meeting, ALS Today talked to Nicholas Maragakis MD to learn more about astrocyte replacement and its potential for people with ALS going forward.


To learn more about astrocyte replacement strategies being developed for people with ALS, check out our report from the 2013 meeting of the International Society for Stem Cell Research (ISSCR), ALS Stem to Stern.

Further reading

Haidet-Phillips, A.M., Gross, S.K., Williams, T., Tuteja, A., Sherman, A., Ko, M., Jeong, Y.H., Wong, P.C. and Maragakis NJ (2013) Altered astrocytic expression of TDP-43 does not influence motor neuron survival.  Experimental Neurology doi: 10.1016/j.expneurol.2013.10.004.  Abstract |  Full Text  (Subscription Required)

Serio, A. et al. (2013) Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.  Proceedings of the National Academy of Sciences 110(12): 4697-4702.   Abstract  |  Full Text