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SHEFFIELD, UK & HAYWARD, Calif., Feb. 13, 2019 (GLOBE NEWSWIRE) – Researchers at the University of Sheffield have identified a new, disrupted pathway in amyotrophic lateral sclerosis (ALS), a debilitating motor neuron and ultimately lethal disease (MND). The discovery was made possible by converting skin cells into brain cells called astrocytes and comparing the energy metabolism of cells extracted from ALS compared to normal controls, using a new metabolic phenotyping technology developed by Biolog , Inc., a California biotechnology company. Scientists have discovered the loss of a key enzyme that could have major consequences on how the central nervous system copes with aging, stress and loss of energy metabolism. The details of the discovery are published in the March issue of the journal "Brain". https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awy353/5303656
ALS, also known as Lou Gehrig's disease, is a disease that appears in adulthood and leads to the loss of motor neurons that control skeletal muscles. At some point in the patient's life, his motor neurons begin to die, leading to muscle wasting or even death, usually within two years of diagnosis. Disruption of the ability of central nervous system cells to produce energy is considered to be a major contributing factor to the disease and may affect rates of disease progression. The disease kills six people every day in the UK and there is no cure.
Sheffield scientists, led by Drs. Scott Allen, Laura Ferraiuolo and Dame Pamela Shaw, used a method previously developed by Drs. In the United States, Ferraiuolo and Meyer take cells from patients' skin, reprogram them into brain cells and watch them to identify new pathways of metabolic dysfunction. This has never been done before. Working in collaboration with the American company Biolog, Inc., world leader in cell badysis, they used a metabolic scanning technology to search for and identify differences in astrocyte metabolism of patients with ALS. Astrocytes are star-shaped brain cells that play a key role in supporting motor neurons by acting as a crucial source of energy nutrients in the central nervous system.
Dr. Allen, a senior scientist at the Sheffield Institute for Translation Neuroscience (SITraN) and funded by the Motor Neurone Disease Association, discovered that cells in ALS patients had a reduced ability to convert a metabolic substrate called adenosine into energy because of of the loss of a key enzyme called adenosine deaminase. The consequence of this loss could be a toxic accumulation of adenosine in the CNS and a subsequent loss of inosine production. Inosine is a metabolic intermediate usually protective of neuronal cells. Until now, the link between the production of ALS and inosine by the loss of adenosine deaminase has not been established. As further confirmation, Dr. Allen fed inosine ALS astrocytes and found that energy production was increasing and that the patient's astrocytes were taking better care of the motor neurons. thus helping them to live longer.
"We are really excited about this set of results because no one has yet been involved with adenosine deaminase in ALS," commented Dr. Allen. "Our results indicate that the higher the level of adenosine deaminase, the higher the protection against adenosine – induced toxicity and the higher the motor neuron support is when inosine is administered. Although we are at an early stage, I think that approaches to increase adenosine deaminase levels, combined with inosine supplementation, could slow the progression of the disease in patients with ALS and improve the quality of life. It has already been shown that altering the level of adenosine deaminase by gene therapy was beneficial and safe for patients with severe combined immunodeficiency. In addition, inosine is a safe and readily available nutritional supplement that has been successfully tested in patients with Parkinson's disease. However, additional laboratory tests will have to be performed. Our donors, Neurocare and the Motor Neurone Disease Association, have given us fantastic support, as well as the technical support of Biolog. I look forward to following these exciting results. "
Barry Bochner, CEO of Biolog, said, "Biolog cell badysis technology has been developed to provide scientists with a new approach to compare normal cells to pathogenic cells to look for subtle differences. underlying deficiencies. We thank Dr. Allen and his colleagues for being one of the first to develop an excellent cell model and then use Biolog technology strategically. "
About Biolog, Inc.
Biolog is a privately held company based in Hayward, Calif., Which continues to play a leading role in the development of powerful new cell badysis tools to solve critical biological, pharmaceutical, and pharmaceutical research and development issues. biotechnology. It is the world leader in phenotypic profiling of cells. In addition to the Phenotype MicroArray ™ product line, the company recently launched a new line of MitoPlate ™ products that can measure more than 50 human cell mitochondrial properties. These, as well as the Phenotype MicroArray tests, can all be read on the versatile OmniLog® instrument from Biolog. Product information and details about MitoPlates are available at http://www.biolog.com/products-static/mitochondrial_function_badays.php. Biolog products are available worldwide, either directly from the company or via its extensive network of international distributors. Additional information can be obtained from the Biolog website, www.biolog.com.
About Sheffield University
The University of Sheffield is one of the largest universities in the world and is part of the prestigious British group Russell, made up of leading institutions led by research. Sheffield has six Nobel Laureates among her former collaborators and students and alumni continue to hold positions of high responsibility and influence all over the world, making a significant contribution in the areas of their choice. Global customers and research partners include Boeing, Rolls-Royce, Unilever, AstraZeneca, GlaxoSmithKline, Siemens and Airbus, as well as numerous UK and foreign government agencies and charitable foundations. Within the University, SITraN is an international center of excellence for basic research and applied research on neurodegenerative diseases. Since it was opened by Her Majesty the Queen in 2010, SITRAN has become a world-leading, state-of-the-art research and know-how, pioneering new treatments for neurodegenerative diseases and bringing new hope to patients and families. from the United Kingdom. The center, worth 18 million pounds sterling, houses state-of-the-art labs and equipment, including a clinical database of over 1,500 patients and a vital resource of human brain bank equipment.
For more information, contact:
Susan Murphy
(510) 785-2564 ext. 360
[email protected]
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