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Birmingham, UK – In a groundbreaking development, researchers from the University of Birmingham have unveiled a novel technique that could significantly advance the understanding of motor neurone disease (MND), also known as amyotrophic lateral sclerosis (ALS). The new approach, announced on Thursday, offers a fresh perspective on the brain and spinal cord changes associated with this debilitating condition.
Motor neurone disease is a progressive neurological disorder that causes muscle wasting and weakness due to the failure of motor neurons in the brain to transmit signals to muscles. Currently, there is no cure for MND, making the search for effective treatments a high priority in medical research.
The newly developed technique, called native ambient mass spectrometry (NAMS), allows scientists to study specific proteins in their native state directly from brain and spinal cord tissue samples. This method marks a significant leap in the ability to observe proteins with exceptional detail, particularly in understanding their structure in relation to their location within tissues.
Using NAMS, the researchers identified a critical metal deficiency in a protein known as superoxide dismutase 1 (SOD1). SOD1 has long been linked to motor neurone disease, but the Birmingham team’s research is the first to demonstrate how metal-deficient versions of this protein accumulate in specific regions of the brain and spinal cord in MND-afflicted mice. This accumulation, they suggest, correlates strongly with the pathology of the disease.
“This approach is the first to show that this form of SOD1 correlates with the pathology of motor neurone disease,” said Helen Cooper, the lead researcher at the University of Birmingham’s School of Biosciences. “It’s a very early step towards finding treatments for MND and is also an exciting new route for understanding the molecular basis of other diseases in unprecedented detail.”
MND, although relatively rare, predominantly affects individuals over 50 years old, though it can develop in adults of any age. The lifetime risk of a person developing MND is approximately one in 300, underscoring the need for ongoing research into its causes and potential treatments.
The next phase of the research will involve testing whether the same metal imbalances identified in the mice are present in human tissue samples. Additionally, the researchers plan to explore potential treatments by attempting to correct these imbalances in mice using existing drug compounds.
If successful, this research could pave the way for new therapeutic strategies, offering hope to those affected by this currently incurable disease.
