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Study identifies key player in motor neuron death in Lou Gehrig's disease
Date:3/26/2014

olved in this disease, so one of our approaches was to find out what weapons these cells might be using to kill motor neurons," Dr. Kaspar says. "And our findings suggest that the microglia utilize an NF-κB-mediated inflammatory response as one of its weapons."

Inhibiting the protein in astrocytes had no impact on disease progression, so the search for the weapons that cell type uses against motor neurons continues. These preliminary findings also don't tell scientists how or why NF-κB turns the ordinarily protective microglia into neuron-killing molecules. But despite the mysteries that remain, the study moves scientists closer to finding a treatment for ALS.

The search for an ALS therapy has been focused in two directions: identifying the trigger that leads to disease onset and understanding the process that leads to disease progression. Changes in motor neurons are involved in disease onset, but disease progression seems to be dictated by changes to astrocytes, microglia and oligodendrocytes. Some cases of ALS are hereditary but the vast majority of patients have no family ties to the disease. The complexity of the disease and the lack of a clear familiar tie make screening before disease onset nearly impossible, highlighting the importance of slowing the disease, Dr. Kaspar says.

"Focusing on stopping the changes that occur in astrocytes and microglia has clinical relevance because most people don't know they're getting ALS, says Dr. Kaspar, who also is an associate professor of pediatrics and neurosciences at The Ohio State University College of Medicine. "We have identified a pathway in microglia that may be targeted to ultimately slow disease progression in ALS and are exploring potential therapeutic strategies and may have broader implications for diseases such as Alzheimer's and Parkinson's Disease amongst others."


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Contact: Gina Bericchia
Gina.Bericchia@NationwideChildrens.org
614-355-0487
Nationwide Children's Hospital
Source:Eurekalert

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