(MEDICAL NEWS) – An international team of investigators has discovered that an inorganic polyphosphate released by nerve cells known as astrocytes in people with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) contributes to the motor neuron death that is the signature of these diseases. The research, by Brigitte van Zundert, PhD, adjunct professor of neurology at UMass Chan and professor at the Universidad Andres Bello in Chile; Robert H. Brown Jr., DPhil, MD, professor of neurology, and colleagues appears this week in Neuron.
"We are encouraged by these early results," said Dr. Brown, the Leo P. and Theresa M. LaChance Chair in Medical Research. "These findings provide an entirely new perspective on ALS pathogenesis, raising exciting hypotheses and possibilities both for disease biomarkers and for therapeutic targets."
ALS and FTD are characterized by the degeneration of motor neurons in the spinal cord and frontal lobes, but the causes of this neurotoxicity have remained elusive. And while great progress has been made in identifying genetic mutations that cause these neurodegenerative disorders, the vast majority of cases have no identifiable genetic mutation. How genetic changes affect neurons, and the possible impact of toxic factors, has also remained elusive.
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