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News from the 2012 ALS/MND International Symposium

Researchers at the Amyotrophic Lateral Sclerosis (ALS) Motor Neuron Disease (MND) International Symposium met for three long and intense days in December 2012 to discuss the newest research on all aspects of ALS.  Below is the summary of one of these important topics. 

Why are some Motor Neurons More Vulnerable than Others?

     Understanding why ocular motor neurons are spared may lead to protective strategies for all motor neurons

Most motor neurons die off in ALS, but some appear to be more resistant to the disease process than others.  That is especially true for the neurons controlling the eye muscles, which remain intact until the end of life in ALS patients.  What accounts for this difference? And can it offer any clues for making other neurons more resistant?

Pamela Shaw, M.D., of the University of Sheffield, United Kingdom, has approached this question by comparing the set of genes used by vulnerable and resistant neurons.  She has found that more than 1700 genes are “differentially expressed,” meaning the resistant neuron used them either significantly more or significantly less than the vulnerable neuron.  The data she collected is available online for other researchers to use freely.  The genes that were most different between them governed such functions as energy management, signaling, and RNA processing.  Some or all of these changes may relate to the ability of resistant neurons to better handle toxic stresses from too much excitation, which is one hypothesized mechanism in the ALS disease process.  Further exploration of these changes may reveal more about possible targets for new treatments.

Jeffrey Macklis, Ph.D., of Harvard University in Cambridge, Massachusetts, studies the development of the nervous system.  In a wide-ranging talk, he pointed out that motor neurons arise as a distinct subset within the nervous system from early in development and that “even subtle abnormalities” in early development of this subset might predispose them as a group to vulnerability and later neurodegeneration.  By better understanding these early developmental events, he hopes to build a more complete understanding of those vulnerabilities, which may suggest ways to strengthen their resistance later in life

More News from the Symposium, visit:

New Understanding of Protein Recycling in ALS: New genes and new pathways reveal potential new targets for treatment

The Roles of Nonneuronal Cells in ALS: Astrocytes and immune cells are shaping up as key players in the ALS disease process

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