RESEARCHERS AT the Royal College of Surgeons in Ireland have discovered how the body protects itself against the onset of motor neuron disease. The discovery immediately opens up new treatment options to counter this deadly disease.
Follow-up research has already shown that symptoms can be reduced and life expectancy can be increased in mice that develop motor neuron disease, said Prof Jochen Prehn, who leads the research at the college.
“It is significantly better than the best current treatment,” said Prof Prehn of the department of physiology and medical physics. “You can significantly delay the onslaught of the symptoms and improve survival.”
The disease is a progressive and fatal degenerative disease where the nerve cells that carry information between muscles and the brain – motor neurons – begin to die. Researchers do not know why this happens, but five years ago Irish geneticists Prof Orla Hardiman and Prof Andrew Green discovered a mutated gene that seemed to be associated with motor neuron disease. The gene produced a protein called angiogenin.
Prof Prehn’s group subsequently focused on angiogenin and became the first to explain how the protein was involved in protecting motor neurons from becoming damaged. They published their research in the current issue of the Journal of Neuroscience.
Prof Prehn described the work related to angiogenin as a “completely Irish” success given the initial discovery and later work happened here. Joint first authors on the study Alexandra Skorupa and Matthew King were based at the college, as was Áine Behan, who was also involved in the research and publication.
If motor neurons break down in a healthy individual, angiogenin is released. It acts like an instant “survival factor”, Prof Prehn said. “The protein machinery is turned on. It helps the surrounding tissue to regenerate or grow new blood vessels or deliver more energy.”
Several factors are released by this machinery, helping to keep the motor neurons healthy so they can continue to support muscle control. Angiogenin in those who develop motor neuron disease is absent or might not work properly.
The prominent cosmologist Prof Stephen Hawking has suffered this loss of motor neurons, and popular RTÉ sports and racing commentator Colm Murray is also struggling with the disease.
Prof Prehn’s group were the first to reveal how angiogenin works to protect the neurons. But this also threw up possibilities for new kinds of treatments.
The researchers plan to study the various protective factors released by the neurons to better understand how they help neighbouring cells, Prof Prehn said.
Funding has come from Enterprise Ireland, Science Foundation Ireland and the Thierry Latran Foundation. There are about 300 people living with motor neuron disease in Ireland at any given time and on average one person is diagnosed every four days.