ALS Latest News And Updates: Scientists Discover Cells That Destroy Motor Neurons In ALS

First Posted: Sep 30, 2016 05:10 AM EDT

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Now, a new discovery of oligodendrocyte in vitro model showed that human cells, which usually support motor neuron function, play a major role in ALS development. Experts believe that the discovery could point toward therapeutic timing and targets.

It may not be a common knowledge for everyone, but ALS has two types: sporadic and familial. The sporadic form of the disease accounts for 90-95 percent of all cases in the United States and happens randomly with no clearly associated risk factors, while familial ALS, which accounts for 5-10 percent of all U.S cases, is inherited.

A report in stated that a lot of the studies in the past have shown that cells which normally help neurons like astrocytes and microglia, can add to motor neuron death during the progression of ALS. Recently, mouse models in the lab showed that oligodendrocytes, also important to normal motor neuron function, also contribute to cell death while ALS progress.

An international team of researchers led by Dr. Laura Ferraiuolo from the Sheffield Institute of Translational Neuroscience (SITraN) at The University of Sheffield made the groundbreaking discovery of how oligodendrocyte brain cell can affect the progression of ALS. The team found that oligodendrocytes - which typically assist with neuron function - can become destructive and cause cell death after developing an innovative oligodendrocyte in-vitro model from the skin cells of people with ALS. "This is the first human in vitro model allowing us to study the specific interaction between neurons and oligodendrocytes from ALS patients," says Dr. Ferraiuolo.

The new study conducted by researchers at Nationwide Children's Hospital is the first study to ever use oligodendrocytes from the skin of human ALS patients to show that these cells can trigger motor neuron death. Medical Xpress reported that the study, published in Proceedings of the National Academy of Sciences, also discovered that depleting the level of SOD1 in early ALS oligodendrocyte progenitors can save motor neurons.

"We were able to dig deep in trying to make a bridge between a mouse model and what is happening in humans," says Brian Kaspar, Ph.D., senior author of the paper and a principal investigator in the Center for Gene Therapy at The Research Institute at Nationwide Children's. "We have been able to begin asking questions about how exactly oligodendrocytes go wrong and how they lead to motor neuron death," reported Medical News Today.

The researchers created a novel co-culture model that enables them to observe mouse and human ALS oligodendrocytes and how it affects the motor neurons. Moreover, the study partly depended on a "direct conversion" method first used in Dr. Kaspar's laboratory which allowed the programming of skin cells from live patients with ALS to become neural progenitor cells.

Results of the study showed oligodendrocytes from both familial and sporadic ALS patients result in motor neuron death as opposed to those with other neuromuscular disorders or healthy people's oligodendrocytes which leave motor neurons safe. Researchers claim that the findings show distinct characteristics of oligodendrocytes from ALS.

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