Transplanting healthy nerve cells could slow ‘Stephen Hawking disease’

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Scientists from University College London (UCL) have found that a motor neuron transplant from a donor can restore muscle function in amyotrophic lateral sclerosis. Work published in the preprint catalog eLife Reviewed Preprint.

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease in adults. These nerve cells provide coordination of movements and maintenance of muscle tone. In ALS, the connections between nerves and muscles are broken, leading to muscle weakness and then paralysis.

The average survival time of this disease after the onset of the first symptoms is about 20-48 months. In rare cases, the disease may become stable. This happened to Stephen Hawking, who has been living with ALS for decades.

The cellular and molecular changes underlying neuronal degeneration in ALS are extremely complex and can vary widely from patient to patient. Therefore, no treatment is currently available that can prevent the progression of symptoms.

In a new study, scientists transplanted healthy motor neurons into the tibial nerve of mice with a highly aggressive model of ALS. The H57-597 antibody was used to suppress the immune system’s response to transplanted cells. This helped prevent graft rejection and successfully repaired some nerve connections to the targeted hind leg muscles. But the muscle contractions were still relatively weak. The fact is that without regular stimulation, transplanted neurons can survive but fail to form mature neuromuscular junctions.

To ensure regular stimulation, the scientists used a wireless system to stimulate neurons with light. At the same time, regular muscle contractions occurred for an hour each day. After 21 days of such “training”, the strength of muscle contraction in animals increased 13 times.

More research is needed to confirm whether the transplant procedure will work on human neurons and whether it will be sufficient to improve a patient’s quality of life.

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