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Abnormal Astroglia

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

Astrocytes help to clear away toxic glutamate from motor neuron synapses. In the mid-1990s, our scientists discovered that astrocytes in many ALS patients and the SOD1 rodent models of the disease  are unable to do this properly due to abnormal transport molecules in the astrocytes.

These astrocytes also contain toxic protein aggregates that increase in number as the disease progresses. Packard studies in animal models have shown that damaged astrocytes increases the vulnerability of motor neurons to damage.

Exactly how astrocytes single out motor neurons for destruction isn’t well understood; it’s likely that the these cells may produce something toxic, as well as fail to clear glutamate away properly. This is an area of intense study.

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Johns Hopkins University
Motor neurons can only work properly if the cell’s proteins can get to the right place at the right time. Thomas Lloyd uses the fruit fly Drosophila melanogaster to study how proteins are shuttled between the cell body and the synapse, as interruptions in this process have been linked to ALS. 
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University of Michigan
Sami Barmada wants to answer a very basic question about ALS: why motor neurons? Of all the different types of neurons in the body (and scientists estimate there are probably several hundred), it’s only motor neurons that are affected in ALS. Knowing why this is, Barmada believes, could be the key to developing new potential treatments that could prevent the deterioration and death of motor neurons. 
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