Causes of ALS: Abnormal Astroglia

A growing number of studies suggest that the processes that damage motor neurons in ALS don’t arise in those cells exclusively. Neighboring astroglial cells, Packard research has shown, contribute to ALS’s progression. 

In the mid 1990s, our scientists discovered that astrocytes in many ALS patients and the SOD1 rodent models of the disease are flawed in clearing toxic glutamate away from motor neuron synapses. The molecules that transport glutamate out of the synapse were found to be abnormal.

The astrocytes also contain unusual aggregations of protein that increase in number as the disease progresses. Packard studies in animal models have shown that the vulnerability of motor neurons to damage is influenced by the cellular environment in general and by having damaged astrocytes in particular.

Astrocytes’ exact role in the singling out of motor neurons for destruction isn’t well understood; it’s likely that the former cells may produce something toxic – as well as fail to clear glutamate away properly. This is an area of intense study.

Learn more about the research projects funded and coordinated by the Packard Center for ALS Research at Johns Hopkins, and targeted at finding the causes of ALS and a cure. Subscribe to ALS Alert to stay informed about ALS research and clinical trials.