Will helping neurons to communicate help alleviate neurodegenerative disease?

A research team led by George Z. Mentis may uncover a new way to fight spinal muscular atrophy (SMA). SMA causes progressive muscle wasting and paralysis, and often leads to death.

Dr. Mentis is associate professor of pathology and cell biology and of neurology at Columbia University Medical Center (CUMC), and a member of the Motor Neuron Center and the Columbia Translational Neuroscience Initiative at CUMC.

Triggered by a genetic mutations that causes a deficiency of a protein called SMN, the disease begins as early as infancy. As levels of SMN drop, motor neurons, which control movement, begin to fail and eventually die. Using mouse models of SMA, Mentis and his colleagues have demonstrated that SMN deficiency in sensory neurons also causes changes in the synapses that connect them to motor neurons. This sets off a chain reaction that diminishes the neurons’ ability to send signals that tell muscles to contract. When the mice were treated with a compound that stimulates synaptic activity, signaling between sensory neurons and spinal motor neurons returned to nearly normal levels, and their motor function improved.

These findings suggest a novel approach to boosting motor neuron health by increasing communication between different types of neurons, which may offer a new avenue for the treatment of SMA. Learn more.

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