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Researchers at the Sheffield Institute for Translational Neuroscience (SITraN) have used a new imaging technique to measure the function of mitochondria in patients with motor neuron disease (MND).
The study, published today in the journal Brain, could provide new ways to evaluate the effectiveness of treatments currently being developed for MND.
The researchers used an advanced imaging technique called 31-phosphorus magnetic resonance spectroscopy to measure chemicals that are critical to energy metabolism in the cell. The study was carried out on patients with MND and on age- and gender-adjusted healthy controls. The patient perceives the procedure as a standard MRI scan, but researchers can measure chemicals directly to get a full picture of the energy status in patients with MND.
MND, or amyotrophic lateral sclerosis (ALS) as it is also known, is a disorder that affects the nerves – motor neurons – in the brain and spinal cord, which form the connection between the nervous system and muscles to allow the body to move. The messages from these nerves gradually fail to reach the muscles and lead to weakening, stiffening and wasting.
There are many barriers to developing effective treatments for MND as we still do not fully understand how or why they develop in the first place. However, there is good evidence from laboratory data and disease models that the mitochondria – or powerhouses of the cell – may be impaired.
The study's lead author, Dr. Matilde Sassani of the University of Sheffield said, "It is important to have an effective technique for measuring mitochondrial function in vivo in patients with MND.
"In this study, we found that brain phosphocreatine levels were decreased compared to healthy controls, and in muscle we found that inorganic phosphates were increased in patients with MND. Both of these findings are consistent with the mitochondrial dysfunction seen in these living people agree with MND. "
Dr. Thomas Jenkins, senior author of the paper and clinical lecturer at SITraN, said, "This research has the potential to influence the development of more effective treatments for MND. It appears to be a promising tool by which to measure the effects of MND drugs, that affect mitochondrial function in people with MND.
"Treatments aimed at saving mitochondrial function in MND are being studied in laboratories around the world. This non-invasive tool can show if drugs in development are successfully targeting mitochondria. This is an important step in choosing Treatments to be used for clinical trials. ""
Future research aims to extend the scope of this study to a larger number of MND patients. Since the mitochondria are believed to be a factor in a number of other neurodegenerative diseases, this technique can potentially be extended to different patient populations.
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University of Sheffield
The imaging technique is proving effective in measuring mitochondrial dysfunction in motor neuron disease (2021 Jan 13).
accessed on January 13, 2021
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