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HSCI researchers identify another potential ALS treatment avenue
Date:8/6/2014

in the nervous system, were involved in motor neuron degeneration in a mouse model of ALS. And the following year the researchers reported that the same thing was happening in human motor neurons made from patient stem cells, and proposed that prostanoid molecules, a group of substances involved in inflammation in everything from pain to pregnancy, might be playing a role in the glial cells.

Today the researchers reported they have confirmed there is a change in prostanoid receptors in the gial cells playing a role in ALS, and with genetic and chemical experiments they showed that this is playing a role in ALS. They further report that when the effected receptor is blocked, the ALS damage done by the glial cells is reduced.

This latest work, says Eggan, first done in human motor neurons in a dish, and then in a mouse model of ALS, "says that indeed this stem cell model was predictive of something that can happen inside a whole animal, and its important because it demonstrates that this is really an important target for an ALS therapeutic. If we can inhibit this receptor in an ALS patient, we might slow down the progression of the disease, and that would be a huge step."

Eggan said "one feature of the glial cells in ALS that attack motor neurons is that they have higher expression of this prostanoid receptor. Removing just one of the two copies of the receptor in the glial cells had an effect on extending the life span" of the ALS mice," Eggan said, and "inhibition by a drug is unlikely to have an effect as complete as a knockout in the mice."

Eggan said that experiments on human stem cell-generated ALS motor neurons also show that "if we inhibit that receptor in the ALS cells with a chemical, those cells lose their toxicity to motor neurons

"This is a very exciting period for those whose lives are threatened by ALS, and it is exciting for my lab," Eggan said. "First we recently identified a pathway that we think is important for degeneration ins
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Contact: B. D. Colen
617-495-7821
Harvard University
Source:Eurekalert  

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