02:46am Tuesday 25 February 2020

Irish Motor Neuron Disease research may lead to new therapies

Motor Neuron Disease (also referred to as Amyotrophic Lateral Sclerosis – ALS) is a progressive and fatal neurodegenerative disease that is caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement, resulting in paralysis and eventual death. Little is know about the causes of Motor Neuron Disease and currently there is no effective cure for the condition.

When motor neuron cells break down, they release the angiogenin protein which protects the remaining motor neuron cells from degrading and enables healthy individuals to maintain control over voluntary muscle movement.

The RCSI researchers have now discovered the mechanisms behind how the angiogenin proteins protect the motor neuron cells. Angiogenin protein is thought to be either absent or ineffective in individuals with Motor Neuron Disease so this research provides insights into how this protein may be used as a therapeutic.

Commenting on the results, lead researcher, Professor Jochen Prehn, Department of Physiology and Medical Physics, RCSI said: “Our study has revealed the mechanisms behind how the angiogenin protein can protect motor neuron cells which is a crucial finding on the path to finding treatments for Motor Neuron Disease.”

“RCSI researchers are now looking at how the angiogenin protein can be used as a theraputic to be used in patients with Motor Neuron Disease and our initial findings have shown that the protein can extend the lifespan of motor neuron cells and improve motor performance,” Professor Prehn concluded.

The joint first authors on the study were Alexandra Skorupa and Dr. Matthew King.

There are approximately 300 people living with Motor Neuron Disease in Ireland at any one time. On average, one person is diagnosed with the disease every four days. (1)

The research, which was recently published in the Journal of Neuroscience (2), is funded by Enterprise Ireland, Science Foundation Ireland and the Thierry Lathran Foundation

(1) Source: Irish Motor Neurone Disease Association

(2) Skorupa A, King MA, Aparicio IM, Dussmann H, Coughlan K, Breen B, Kieran D, Concannon CG, Marin P, Prehn JH. Motoneurons secrete angiogenin to induce RNA cleavage in astroglia. J Neurosci. 2012 Apr 11;32(15):5024-38.

Royal College of Surgeons in Ireland, 123 St Stephens Green, Dublin 2, Ireland.  Tel: +353 1 402 2100

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